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Some skin adnexal tumors display both epithelial and myoepithelial cell populations and can be broadly categorized as biphasic tumors. These include apocrine hidrocystoma, mixed tumor, adenomyoepithelioma (AME), and adenoid cystic carcinoma (ACC). Myoepithelioma is the myoepithelial cell-predominant type in this category. Cutaneous AME is exceedingly rare and usually has a benign prognosis, but it is considered to have the potential for local recurrence and metastasis. We report the case of a 57-year-old man with a 1-year history of an ulcerated nodule on his scalp. Microscopically, it was a defined cutaneous nodule with a focal lobulated architecture, composed of epithelial cells forming ducts and myoepithelial cells distributed around the ducts. In addition to these findings of typical AME, the present case focally revealed atypical features, such as increased mitotic activity (7/10 high power fields), invasive growth, and necrosis. However, cytological atypia was not significant. We conclusively diagnosed cutaneous AME with atypical features, suggesting malignant potential. Moreover, areas showing appearances similar to apocrine hidrocystoma, mixed tumor, myoepithelioma, and ACC were focally observed. We present a unique case of cutaneous AME exhibiting histopathological heterogeneity. The recognition of morphological variation could be helpful in appropriately diagnosing and treating AME of the skin.

Purpose: Chondroid syringoma of the skin is a rare subcutaneous tumor and localization in the eyelid and orbital region that has rarely been described. We report a case of chondroid syringoma that involved the sub-brow region and was accompanied by hair loss. Case summary: A 57-year-old women presented with a mass on the lateral side of the left sub-brow region which was observed 25 years earlier. The mass, which recurred 2 months after surgery at another hospital through a skin incision was accompanied by an itching sensation. The mass was not tender or ulcerated but was reddish with superficial blood vessels and had a smooth surface with hair loss at the site of the mass. The mass showed high signal intensity on a T2-weighted magnetic resonance image and a round echogenic nodule with an irregular hypoechoic portion was observed on ultrasonography. A full-thickness excision including the adjacent normal tissue of the sub-brow mass and direct closure were subsequently performed. The pathological diagnosis was chondroid syringoma which was revealed as numerous tubular structures with various lumens in a collagenous stroma. Mucinous and fibrous findings were also observed. No recurrence was detected during the first 2 years after surgery. Conclusions: Chondroid syringoma in the eyelid and sub-brow region is uncommon. Complete resection is required to differentiate it from a malignancy and reduce the possibility of recurrence. Incomplete resection or capsular rupture during removal of the tumor could induce recurrence or a malignant change in the tumor.

Background: Rabbits, ferrets, and four-toed hedgehogs are popular exotic pets, but comprehensive epidemiological studies in these animals have been rarely conducted. The present study aims to clarify the incidence of neoplastic and non-neoplastic diseases in pet rabbits, ferrets and four-toed hedgehogs in Japan. Methods: We histologically/cytologically investigated 1098 samples from 883 rabbits, 812 samples from 521 ferrets and 561 samples from 468 four-toed hedgehogs that were collected at Miwa Exotic Animal Hospital and submitted to the Laboratory of Veterinary Pathology, The University of Tokyo between 2006 and 2020. The examinations of necropsy samples were not included in the present study. Results: Of the 1098 samples from diseased rabbits, 721 (65.7%) were diagnosed as neoplastic and 377 (34.3%) were as non-neoplastic. Uterine adenocarcinoma (21.1%), cutaneous soft tissue sarcoma (15.1%) and mammary gland adenocarcinoma (8.2%) were the most commonly encountered neoplasms. Endometrial hyperplasia (35.8%), testicular atrophy (4.2%) and uterine adenomyosis (3.7%) were the most common non-neoplastic lesions in rabbits. Of the 812 samples from diseased ferrets, 487 (60.0%) were diagnosed as neoplastic and 325 (40.0%) were as non-neoplastic. Adrenocortical tumor (23.2%), lymphoma (19.3%) and pancreatic islet cell tumor (11.5%) were the most commonly encountered neoplasms. Extramedullary hematopoiesis in the spleen (10.5%), lymph node hyperplasia (7.7%) and cholangiohepatitis (4.6%) were the most common non-neoplastic lesions in ferrets. Of the 561 samples from diseased hedgehogs, 338 (60.2%) were diagnosed as neoplastic and 223 (39.8%) were as non-neoplastic. Endometrial stromal tumor and endometrial mixed tumor (23.7%), oral squamous cell carcinoma (13.0%) and cutaneous soft tissue sarcoma (11.5%) were the most commonly encountered neoplasms. Gingivitis/stomatitis (39.5%), endometrial hyperplasia (18.8%) and dermatitis (6.7%) were the most common non-neoplastic lesions in hedgehogs. Conclusions and clinical relevance: Information obtained from the present study will provide a useful reference for veterinarians working with these exotic animals. Based on the literature search, this is the largest-scale retrospective study on disease incidence in hedgehogs.

Introduction Mixed tumor in the same lymph nodes is extremely rare and no previous reports have described mixed tumor comprising urothelial carcinoma and malignant lymphoma. Case presentation A 71-year-old woman visited a local clinic with a main complaint of hematuria. Imaging revealed right hydronephrosis and a mid-ureter tumor shadow. Positron emission tomography-computed tomography showed high uptake of fluorodeoxyglucose in para-aortic lymph nodes. Abdominal para-aortic lymph node biopsy was performed. Pathology showed urothelial carcinoma and malignant lymphoma in the same lymph nodes, where a mixed tumor was diagnosed. Conclusion We encountered a case of mixed tumor of urothelial carcinoma and Hodgkin lymphoma, which metastasized to the same tissues.

Although immunotherapy has achieved great clinical success in clinical outcomes, especially the anti-PD-1 or anti-PD-L1 antibodies, not all patients respond to anti-PD-1 immunotherapy. It is urgently required for a clinical diagnosis to develop non-invasive imaging meditated strategy for assessing the expression level of PD-L1 in tumors. In this work, a 68Ga-labeled single-domain antibody tracer, 68Ga-NOTA-Nb109, was designed for specific and noninvasive imaging of PD-L1 expression in an MC38 tumor-bearing mouse model. Comprehensive studies including Positron Emission Tomography (PET), biodistribution, blocking studies, immunohistochemistry, and immunotherapy, have been performed in differences PD-L1 expression tumor-bearing models. These results revealed that 68Ga-NOTA-Nb109 specifically accumulated in the MC38-hPD-L1 tumor. The content of this nanobody in MC38 hPD-L1 tumor and MC38 Mixed tumor was 8.2 ± 1.3, 7.3 ± 1.2, 3.7 ± 1.5, 2.3 ± 1.2%ID/g and 7.5 ± 1.4, 3.6 ± 1.7, 1.7 ± 0.6, 1.2 ± 0.5%ID/g at 0.5, 1, 1.5, 2 hours post-injection, respectively. 68Ga-NOTA-Nb109 has the potential to further noninvasive PET imaging and therapy effectiveness assessments based on the PD-L1 status in tumors. To explore the possible synergistic effects of immunotherapy combined with chemotherapy, MC38 xenografts with different sensitivity to PD-L1 blockade were established. In addition, we found that PD-1 blockade also had efficacy on the PD-L1 knockout tumors. RT-PCR and immunofluorescence analysis were used to detect the expression of PD-L1. It was observed that both mouse and human PD-L1 expressed among three types of MC38 tumors. These results suggest that PD-L1 on tumor cells affect the efficacy, but it on host myeloid cells might be essential for checkpoint blockade. Moreover, anti-PD-1 treatment activates tumor-reactive CD103+ CD39+ CD8+T cells (TILs) in tumor microenvironment.

Introduction: Pleomorphic adenomas are the most common non-malignant neoplasms of salivary gland tumours that present mixed histopathological patterns. The purpose of our study was to report an unusual case of a pleomorphic adenoma which was located in the upper lip that had been initially considered as cyst. Case report: The patient was a middle aged man who had a painless, slow-growing swelling in the upper lip. At first, the round-shaped and mobile mass was considered as cystic lesion. However during the operation, it was discovered instead of a cyst, an encapsulated solid tumour that demonstrated both epithelial and mesenchymal cells in histopathology and the diagnosis of pleomorphic adenoma was set. One year postoperatively the patient had no recurrence of tumour. Conclusions: Occurrence of intraoral lip swelling could be attributed, even rarely, to benign mixed tumours. Despite its nonmalignant features, these masses have a relatively low but still existing risk for recurrence or malignant transformation and thus in similar cases a systematic follow-up should be performed even years after surgery.

A primordial odontogenic tumor (POT) is a rare benign mixed epithelial and mesenchymal odontogenic tumor that was included for the first time in the current World Health Organization (WHO) classification for head and neck tumors in 2017. Only seventeen cases of this tumor have been reported to the moment of writing this paper. We report a case of POT in a 13-year-old male patient presented with a painless enlargement of maxillary well-defined unilocular radiolucent lesion. Histologically, the tumor consisted mainly of loose fibrous connective tissue which resembles the dental papilla, and a single layer of columnar epithelial cells that covers the periphery of the tumor. Therefore, the histopathological diagnosis of POT was established. Immunohistochemical evaluation revealed a positive cytokeratin (CK) 19, and vimentin expression indicating a mixed tumor with a degree of differentiation corresponds approximately to that of the early bell stage of normal tissue from primary tooth germs.

Chondroid syringoma (CS) represents the cutaneous counterpart of mixed tumor (pleomorphic adenoma) of salivary glands. Definitive diagnosis is made on histopathology and is based on the presence of characteristic epithelial and stromal components. We report a case of an atypical CS arising on the extremity of an elderly male patient. Histomorphologic features of necrosis and cellular atypia raised suspicion for malignant degeneration, an exceptionally rare circumstance in this context. To further support the diagnosis of malignancy, array comparative genomic hybridization was performed from both low and higher grade areas of the tumor. Both regions demonstrated multiple copy number gains and losses, with additional loss of q7p (TP53), loss of 19p, and loss of heterozygosity on16q demonstrated in the more atypical foci. To our knowledge, this is the first case description of malignant degeneration of a CS with correlative microarray analysis. The findings in this case may prove useful in confirming the diagnosis in future ambiguous cases.

An immature teratoma is a teratoma that is known to contain immature anaplastic elements and is often known as malignant teratoma. Around 95% of testicular cancer is germ cell tumor (GCT). GCT is further divided into Non seminomatous GCT (NSGCT) and Seminoma. NSGCT include choriocarcinoma, embryonal carcinoma, yolk sac tumor, teratoma and mixed tumor. Among these only 2–6% of teratoma is pure teratoma. Pure teratoma can be subdivided into prepubertal and post pubertal. Among these two subgroups, the prognosis is also different. We report here a rare case of pure post-pubertal immature teratoma.

Breast cancer is one of the most frequently diagnosed cancers. Although biomarkers are continuously being discovered, few specific markers, rather than classification markers, representing the aggressiveness and invasiveness of breast cancer are known. In this study, we used samples from canine mammary tumors in a comparative approach. We subjected 36 fractions of both canine normal and mammary tumor plasmas to highperformance quantitative proteomics analysis. Among the identified proteins, LCAT was selectively expressed in mixed tumor samples. With further MRM and Western blot validation, we discovered that the LCAT protein is an indicator of aggressive mammary tumors, an advanced stage of cancer, possibly highly metastatic. Interestingly, we also found that LCAT is overexpressed in high-grade and lymph-node-positive breast cancer in silico data. We also demonstrated that LCAT is highly expressed in the sera of advanced-stage human breast cancers within the same classification. In conclusion, we identified a possible common plasma protein biomarker, LCAT, that is highly expressed in aggressive human breast cancer and canine mammary tumor. [BMB Reports 2020; 53(12): 664-669].

Historically USPIO has been used to help with nodal staging but not in primary tumors. The ability to concentrate USPIO may help to differentiate BMT from other types of parotid tumors.

Purpose The TELEACE study showed reductions in tumor size in patients with neuroendocrine tumors, receiving telotristat ethyl in US clinical practice. Here, we report progression-free survival, time to tumor progression, changes in carcinoid syndrome symptoms, and indictors of overall health. Patients and methods This was a retrospective, single arm, pre-post medical chart review of patients with locally advanced or metastatic neuroendocrine tumors and documented carcinoid syndrome receiving telotristat ethyl for at least 6 months. Patients with poorly differentiated tumors, mixed tumor types or conflicting clinical trial enrollment were excluded. Descriptive statistics, Kaplan-Meier and chi-square tests were used to evaluate PFS, tumor progression, changes in symptoms, body weight and ECOG performance status before and after telotristat ethyl initiation. Subgroup analyses were conducted in patients with the same pre- and post-telotristat ethyl background treatment. Results Anonymized data for 200 patients were provided by 114 physicians; patients received telotristat ethyl for a median of 9 months. Median time to tumor progression was 39.8 months (IQR, 18.7-39.8); most had no tumor progression at 6 (92%) and 12 months (87%). Median progression-free survival was 23.7 months (17.8-39.8); most had progression-free survival at 6 (90%) and 12 months (80%). Results were consistent in the subgroup of 65 patients with the same pre/post background treatment. Nearly all patients had improved carcinoid syndrome symptoms, stable or improved weight and ECOG performance status. Conclusion Patients showed improvements in clinical outcomes and indicators of overall health following treatment with telotristat ethyl in this exploratory pilot study, consistent with previously observed reductions in tumor size.

Parachordoma is a rare soft tissue mixed tumor, associated with soft tissue myoepithelioma. It is typically growing slowly and considered less aggressive than other similar soft tissue tumors. However, it does recur sporadically, and on rare occasions, it has demonstrated the ability to metastasize. Although imaging is important, definitive diagnosis is achieved by histology, and it is typically treated by a wide local excision. We present the first reported case of a skull base parachordoma in a 15-year-old boy, managed with a wide local excision and with no signs of recurrence or metastases after 24 months of follow-up.

Introduction Multinodular goiter is defined as multiple discrete nodules in the thyroid gland. The incidence of Papillary carcinoma thyroid was found to be highest out of total Multinodular Goiter cases while that of Anaplastic carcinoma was the least. We report a rare coexistence of Papillary carcinoma and Anaplastic carcinoma in adult patient with a long-standing Multinodular Goiter. Case presentation Here we present a case of 54 years male with huge anterior neck swelling for 20 years with a gradual increase in size. Computerized tomography of neck revealed solidocystic mass lesion without any significant lymphadenopathy, features suggesting Multinodular goiter with differential diagnosis of Carcinoma Thyroid. Cytological examination showed Papillary thyroid Carcinoma. He underwent total thyroidectomy with central neck dissection. Postoperative period was uneventful. Histopathological report revealed features suggestive of mixed tumor of Papillary thyroid Carcinoma and Anaplastic Carcinoma thyroid TNM Staging T3 N0 M0, Stage IVA. After the final reports patient was sent for adjuvant therapy three weeks later where he received megavoltage external beam radiation and he was followed up till 12th week. He was assessed radiologically which showed no signs of physical progression of the disease. However, he was lost to follow up after that visit. Discussion Long-standing benign conditions of thyroid can transform into malignant forms in the undefined duration of time. Conclusion Regular follow up and early management of multinodular goiter at the right time can save a patient from undue stress and complication like the conversion into malignancy.

Obesity may be associated with the onset of mammary tumors in women. In companion animals, these data are still scarce, mainly associating the clinic of the patient with laboratory and histological findings. This study aimed to evaluate the clinical and laboratory aspects of female dogs with mammary neoplasia, investigating whether they are related to obesity. Four groups of spayed or non-spayed female dogs were studied, divided into (G1) female dogs without mammary tumor and normal body condition score (BCS), (G2) female dogs without mammary tumor and obese, (G3) female dogs with mammary tumor and normal BCS and (G4) female dogs with mammary tumor and obese. BCS, canine body mass index (CBMI), blood count, glucose, total cholesterol, triglycerides and cytopathological and histopathological examinations were evaluated. Mixed-breed animals were the most prevalent in this study (67.5%). There was a limitation regarding the use of CBMI related to body proportionality. Considering the 28 tumors evaluated, carcinoma in mixed tumor was the most prevalent malignant histological type, while adenomyoepithelioma was the most prevalent benign histological type. It was possible to conclude that obesity was not related to clinical and laboratory changes in the female dogs affected with mammary neoplasias. In addition, no relationship was noted between BCS and CBMI with the histological type or malignancy of mammary neoplasia, since the malignant histological types were the most prevalent tumors in both the ideal score group and the overweight to obese group. RESUMO: A obesidade pode estar associada ao aparecimento de tumores mamários em mulheres. Em animais de companhia, esse dado ainda é escasso, principalmente associando a clínica da paciente aos achados laboratoriais e histológicos. Este estudo objetivou avaliar os aspectos clínicos e laboratoriais de cadelas com neoplasia mamária, investigando se estão relacionadas à obesidade. Foram estudados quatro grupos de cadelas castradas ou não castradas, divididas em (G1) cadelas sem tumor de mama e Escore de Condição Corporal normal (BCS), (G2) cadelas sem tumor de mama e obesa, (G3) cadelas com tumor de mama e ECC normal e (G4) cadelas com tumor de mama e obesa. Foram avaliados o Escore de Condição Corporal (ECC), Índice de Massa Corporal Canina (IMC), hemograma, glicose, colesterol total, triglicerídeos e exames citopatológicos e histopatológicos. Os animais de raça mista foram os mais prevalentes neste estudo (67,5%). Houve uma limitação quanto ao uso do IMC relacionado à proporcionalidade corporal. Considerando os 28 tumores avaliados, o carcinoma em tumor misto foi o tipo histológico maligno mais prevalente, enquanto o adenomioepitelioma foi o tipo histológico benigno mais prevalente. Foi possível concluir que a obesidade não esteve relacionada a alterações clínicas e laboratoriais nas cadelas acometidas por neoplasias mamárias. Além disso, não foi observada relação entre BCS e CBMI com o tipo histológico ou malignidade das neoplasias mamárias, uma vez que os tipos histológicos malignos foram os tumores mais prevalentes no grupo escore ideal e no grupo com sobrepeso e obesidade.

Desmoplastic melanoma commonly occurs on the head and neck in a pure form, but occasionally, it occurs in a mixed tumor with another type, usually superficial spreading melanoma (SSM), and rarely as a metastasis from a primary SSM. We report here a primary SSM on the leg of a 32-year-old male which metastasised to lymph nodes, and 10 years later recurred at the primary site initially with mixed features but evolving to resemble a uniformly desmoplastic, deeply invasive melanoma. This unusual case has implications for clinical management and is additionally notable for its reversal in behavior, from metastatic to local infiltrative type, correlating with the change in morphology.

Chondroid syringoma - also known as mixed tumor of the skin - is a benign skin tumor often occurring in the head and neck region, that usually presents as a slow growing well-defined swelling in adults, with occasional commitment of the lips. The aim of this study was to report an illustrative case of chondroid syringoma in the lower lip of a 43-year-old male patient from Brazil. Physical examination revealed an asymptomatic hardened swelling of normal coloration, measuring 3.0 cm, located at the transition between the skin and semi-mucosa of the lower lip. Under the clinical hypothesis of a salivary gland tumor, an excisional biopsy was performed under local anesthesia. After histopathologic examination the final diagnosis was of chondroid syringoma of the lower lip. There is no evidence of recurrence one year after surgical procedure. Clinicians should consider chondroid syringoma when evaluating well-defined swellings of the lower lip.

Pleomorphic adenoma (PA), also called mixed tumor, is the most common benign tumor of the salivary glands that mostly occurs in the parotid or submandibular glands but may also occur in the minor salivary glands that are distributed throughout the oral cavity. The common sites of PA of the minor salivary glands are the palates followed by lips and cheeks. Surgical removal with adequate margins is the principal treatment. We present a case of PA (arising from the minor salivary gland of the upper lip) - a rare site in a 65-year-old female who presented with a swelling upper lip of 2-year duration. Fine-needle aspiration cytology of the swelling revealed PA which was confirmed by histopathological examination after complete excision of the swelling.

Mixed tumor of the skin (MTS) is a rare skin adnexal neoplasm occurring in sweat glands. It is usually benign, measures 0.5 to 3 cm, and presents as a slowly growing, painless, firm nodule commonly in the head and neck regions. Owing to its rarity and lack of distinctive clinical manifestations, confirmative diagnosis is made on the basis of its pathologic features. Malignant MTS also develops de novo or from preexisting benign MTS even though they occur rarely. It should be excised completely to exclude malignant potentials. Herein, we report a 35-year history of a giant MTS of eccrine type measuring approximately 10.5 × 6.5 cm on the right hemiface of a 91-year-old woman, which is the largest facial MTS reported in the literature so far, to the best of our knowledge.

Triple-negative tumors are characterized immunohistochemically by the absence of positivity to sex hormone receptors and to human epidermal growth factor receptor 2. Additionally, they are differentiated into basal-like and non-basal (or null) subtypes, based on the presence of basal cytokeratin expression (CK5/6, 14, and17). Triple-negative subtypes are yet to be characterized in male dogs, to our knowledge. We report herein the clinical and pathologic findings and molecular characterization of carcinoma in the mammary glands of 2 male dogs. Case 1 was diagnosed as a grade II tubulopapillary carcinoma; case 2 was diagnosed as a grade II carcinoma in a mixed tumor. The tumors were characterized phenotypically as triple-negative basal and triple-negative non-basal, respectively.

Adenoid basal carcinoma (ABC) is uncommon malignancy of the uterine cervix and it can be pure or combined with cervical intraepithelial lesions. There were less than 20 cases of ABC combined with invasive squamous carcinoma (mixed type) in English literature. These cases had similar properties as seen at postmenopausal women and diagnosed with abnormal cervical smear findings. Here we present a case of 37-year-old woman who suffered from spotting and received endocervical curettage. The pathological report revealed squamous cell carcinoma (SCC) of the cervix. The patient underwent type 3 radical hysterectomy and bilateral pelvic and para-aortic lymph node dissection. The final pathological report revealed SCC coexisting with ABC. Human papillomavirus (HPV) 16,18 and others (11 types) were negative in both components of the mixed tumor by in situ hybridization detection. Our case was cytokeratin 7 negative, cytokeratin 8 positive and p63 positive which supports the hypothesis that mixed type cervical carcinoma originates from endocervical reserve cells.

Introduction Soft tissue myoepithelioma (STM), a rare mesenchymal neoplasm morphologically analogous to its more common salivary gland (SG) counterpart, is the subject of single case reports regarding its fine-needle aspiration (FNA) biopsy. To our knowledge, ours is the first case series of STM. Materials and methods A search was made of our pathology databases for cases diagnosed as STM. FNA biopsy smears and cell blocks were performed using standard techniques. Results Seven cases were retrieved from 4 men and 3 women (M:F = 1.3:1; age range: 25-79 years, x = 54 years). All but 1 presented as a primary neoplasm. Six aspirates were from the extremities, and 1 from the abdominal wall. Mean tumor size was 5.7 cm. Cytologic diagnosis of STM or suspicious for STM was made in 3 cases (43%). Remaining FNA diagnoses were spindle cell neoplasm/lesion (2), spindle cell sarcoma (1), and extraskeletal myxoid chondrosarcoma (1). Three cases were composed primarily or solely of uniform spindle cells, 3 primarily of uniform epithelioid cells with plasmacytoid features, and 1 case a mixture of these 2 cell types. Myxoid/chondromyxoid stroma was relatively abundant except in the single hypocellular example. Immunohistochemical (IHC) testing performed in 71% was nonspecific, but positive with S-100 in 4 of 5, EMA in 3 of 3, calponin in 2 of 2, and keratin in 1 of 3 examples. Conclusion FNA biopsy smears of STM are remarkably similar cytomorphologically to their SG equivalent. However, STM can be misidentified principally as extraskeletal myxoid chondrosarcoma, thus requiring a relatively broad IHC panel for a specific diagnosis.

Mixed epithelial and stromal tumor (MEST) of the kidney is a rare tumor composed of epithelial and stromal cells. Steroid hormones seem to have a role in genesis and evolution of MESTs family, considering the marked female predominance, common history of long-term estrogen replacement, the frequent expression of estrogen (ER) and progesterone (PR) receptors in tumor mesenchymal component. We report the case of a 37-year-old woman admitted to the Department of Surgery because of abdominal pain. She had taken oral estrogen-progestin therapy for 4 years until the age of 35 for dysmenorrhea. During an abdominal ultrasound (US) was identified a left ovarian cyst, 60 mm x 20 mm, suggestive of simple cyst. Then a US repeated after 12 months showed the enlargement of the ovarian cyst (76 mm × 53 mm x 78 mm) and the appearance of a new exophytic cystic-solid mass of 120 mm in the lower pole of the left kidney. The patient underwent laparoscopic left partial nephrectomy and left ovariectomy. Histopathological examination and immunohistochemical analysis was consistent with a MEST of the kidney and a primary serous cystadenoma of the ovary. We report the second case in the literature of a MEST of the kidney along with a primary serous ovarian cystadenoma. We emphasize the hypothesis of the correlation between MESTs and hormonal milieu and the importance of clinical evaluation prior to the administration of any hormone therapy.

Hepatocellular carcinoma (HCC) with the concurrent occurrence of primary hepatic neuroendocrine carcinoma (NEC) of the liver is extremely rare. Preoperative diagnosis of HCC combined with NEC is very difficult. Here, we presented a case report of HCC combined with NEC. A 33-year-old male was admitted to our hospital due to focal liver lesion. To further the diagnosis, he received laboratory tests, conventional ultrasound, contrast-enhanced ultrasound (CEUS), and magnetic resonance imaging (MRI). Grayscale ultrasound showed a hypoechoic nodule with peripheral hypoechoic halo and central small patches of anechoic area in the hepatic segment VI, and the liver background was cirrhosis. In the CEUS, the solid component of the nodule was rapidly homogeneous hyper-enhancement in the arterial phase. Then, the enhancement of the nodule was washed out slowly and gradually; the nodule presented heterogeneously mild enhancement in the portal phase, and slight hypo-enhancement was showed in the late phase. The hypo-enhanced area was mainly located in the surrounding space. Meanwhile, there was a small piece of non-enhanced area within the lesion center in the whole three-phase imaging. MRI showed a lobular contoured mass in segment VI. The patient underwent middle liver resection, splenectomy, and cholecystectomy. The pathological diagnosis was a HCC with NEC. At the time of the preparation of this manuscript, the patient has been alive without recurrence or distant metastases for 6 months since the surgery. We mainly focus on the ultrasound imaging characteristics, especially its enhancement manifestations on CEUS. In this report, since this article is a case report, which is based on the clinical information of the patient and does not involve the patient's privacy, informed consent is not necessary. In addition, the patient agreed to publish the case. To the best of our knowledge, this report is the first to describe the CEUS patterns of the HCC combined with NEC. Herein, we report a case that provides novel insights that will improve clinicians' awareness of the clinical and ultrasound manifestations of this mixed tumor, resulting in improved diagnosis, treatment, and outcomes.

Chondroid syringoma is a benign, rare, asymptomatic, slow-growing mixed tumor. Tumors larger than 3cm are considered giant and may be malignant. We present a 65-year-old man with a chronic, indolent, subcutaneous tumor with upper lip deformity. An excisional biopsy was performed under local anesthesia. Based on histopathological analysis, the resected lesion was identified as a chondroid syringoma of the apocrine type. There was no recurrence during the one-year follow-up after surgery. The occurrence of a large chondroid syringoma in the upper lip is rare.

Mixed adenoneuroendocrine carcinoma (MANEC) of the gastrointestinal (GI) tract is a rare subtype of mixed tumors, and it is scarcely described in the literature. MANEC tumors are composed of adenocarcinoma and neuroendocrine carcinoma components, each of which comprises at least 30% of the lesion. Diagnosing MANEC requires specific histological and immunohistochemistry (IHC) analysis. Typically, MANEC tumors carry a poor prognosis due to their very aggressive nature. We report the case of a 70-year-old female patient with no past medical history who presented with a three-week history of abdominal pain and one episode of hematemesis one week prior to presentation. Initial CT of the abdomen showed a large, 8 x 6 x 6-cm mass arising from the stomach and extending to the lesser sac as well as the central crus of the diaphragm with bilateral retroperitoneal lymphadenopathy. Upper endoscopy revealed an excavated, ulcerated, and partially necrotic mass on the lesser curvature of the proximal gastric body. Tissue biopsy of the lesion showed infiltrating mixed poorly differentiated adenocarcinoma and neuroendocrine carcinoma. On IHC, the adenocarcinoma component stained positively for CDX2 and pancytokeratin, and the neuroendocrine component stained positively for synaptophysin and chromogranin. Further workup included CT of the chest, which demonstrated extensive bilateral pulmonary emboli and new liver lesions with moderate ascites not seen on the initial abdominal CT. The latter was repeated and showed remarkable enlargement of the gastric mass (up to 12 cm) with extensive retroperitoneal adenopathy and mesenteric implants. Given the rapid clinical deterioration and progression of tumor burden, comfort measures were offered and the patient passed away soon after. MANEC tumors are highly aggressive subtypes of "collision" tumors, which are not well described in the medical literature due to their rarity. The etiology is poorly understood with various theories proposing different pathophysiological mechanisms. Standard therapy is not well developed at present; however, a few reports have demonstrated successful outcomes with surgery or combined chemotherapy (cisplatin with irinotecan or etoposide) if diagnosed at an early stage.

Chondroid syringoma is a rare, benign, appendegeal neoplasm. It was initially termed as mixed tumor as it comprises both epithelial cells and chondromyxoid stroma. It usually presents as a slow growing, solitary, painless, subcutaneous, or intracutaneous mass, frequently in the head and neck region. Cytological features usually include the presence of both components, similar to histology but aspiration of only one component or atypical features can pose challenges in diagnosis. According to literature, only a few single case reports describing the cytological features of chondroid syringoma has been published. We report three cases of chondroid syringoma and its differential diagnosis on cytology.

Patient: Female, 58-year-old Final Diagnosis: Mixed adenoneuroendocrine carcinoma of the gallbladder Symptoms: Abdominal pain • asthenia Medication: — Clinical Procedure: — Specialty: Surgery Objective: Rare disease Background: Mixed adenoneuroendocrine carcinoma of the gallbladder (gMANEC) is an extremely rare cancer. Most of the cases are reported in Asia, North America, and Europe, with no cases reported in Latin America; this is the first report for this region, and the 24th case reported worldwide. Case Report: A 68-year-old woman was referred to our department due to asthenia and moderate abdominal pain in the right upper quadrant for 6 months, with imaging examinations showing a solid heterogeneous expansive lesion in gallbladder topography and segment IV of the liver. The MRI displayed an expansive and heterogeneous lesion with inaccurate limits in the gallbladder affecting segment IVb of the liver, in addition to lymphadenopathy in the hepatic hilum. A cholecystectomy with resection of segments IV-B and V of the liver (radical cholecystectomy) and hepatic hilar lymphadenectomy were performed. Anatomopathological examination and immunohisto-chemistry confirmed a primary mixed adenoneuroendocrine carcinoma of the gallbladder. The patient received adjuvant chemotherapy and radiotherapy; however, after the patient reported experiencing low back pain, a CT was performed, revealing retroperitoneal metastasis, and the radiotherapy was interrupted. Currently, the patient has a stable disease, following a protocol of 5-Fluorouracil and somatostatin, and she reports having low back pain of low intensity. Conclusions: This is the 24th gMANEC case reported in the literature. The tumor was successfully resected; however, the patient presented retroperitoneal metastasis 6 months after surgery, despite combined adjuvant therapy.

International audience; Hepatocholangiocarcinoma, fibrolamellar carcinoma, hepatic haemangioendothelioma and hepatic angiosarcoma represent less than 5% of primary liver cancers. Fibrolamellar carcinoma and hepatic haemangioendothelioma are driven by unique somatic genetic alterations (DNAJB1-PRKCA and CAMTA1-WWTR1 fusions, respectively), while the pathogenesis of hepatocholangiocarcinoma remains more complex, as suggested by its histological diversity. Histology is the gold standard for diagnosis, which remains challenging even in an expert centre because of the low incidences of these liver cancers. Resection, when feasible, is the cornerstone of treatment, together with liver transplantation for hepatic haemangioendothelioma. The role of locoregional therapies and systemic treatments remains poorly studied. In this review, we aim to describe the recent advances in terms of diagnosis and clinical management of these rare primary liver cancers.

Pleomorphic adenoma is a benign mixed tumor, which is composed of myoepithelial and epithelial cells. A fibrous capsule separates these cells from the surrounding tissues. Pleomorphic adenoma is the most common salivary gland tumour accounting for 40-70% of all major and minor salivary gland tumours. It is also the commonest minor salivary gland benign tumours accounting for 70% of all tumours. Hard palate is the commonest site followed by upper lip, buccal mucosa, tongue, floor of mouth, retromolar trigone. This case report discusses a case of pleomorphic adenoma of hard palate in an old man after complete excision of the tumour, which was confirmed by a biopsy specimen.

ObjectivePleomorphic adenomas (PAs) with divergent clinical behavior, differing from the vast majority of PAs, were distinguished. “Fast” PAs are characterized by an unexpectedly short medical history and relatively rapid growth. The reference group consisted of “slow” PAs with very stable biology and long-term progression. We divide the PA group as a whole into three subsets: “fast,” “normal,” and “slow” tumors. Our goal is a multifactorial analysis of the “fast” and “slow” PA subgroups.MethodsConsecutive surgeries in a tertiary referral center, the Department of Otolaryngology and Laryngological Surgery, Poznan University of Medical Sciences, Poland, were carried out between 2002 and 2011. Out of 1,154 parotid tumors, 636 (55.1%) were PAs. The data were collected prospectively in collaboration with the Polish National Registry of Benign Salivary Gland Tumors. The main outcome measure was the recurrence rate in “fast” and “slow” PA subgroups. All surgical qualifications and surgeries were performed by two experienced surgeons.ResultsSlow PAs, compared to fast PAs, presented in older patients (53.25 ± 15.29 versus 47.92 ± 13.44 years). Multifactor logistic regression analysis with recurrence (yes/no) as the outcome variable, fast/slow as the predictor variable and age, gender, margin, FN status as covariates showed that fast PAs were significantly predicting recurrence vs. slow PAs (p = 0.035). Fast PAs were increasing the risk of PAs 10-fold vs. slow PAs, exp β = 10.20, CI95 [1.66; 197.87]. The variables impacting relapse were recent accelerated growth of the tumor OR = 3.35 (SE = 0.56), p = 0.030, positive margins OR = 7.18 (SE = 0.57), p < 0.001, incomplete or bare capsule OR = 9.91 (SE = 0.53), p = 0.001 and location III OR = 3.12 (SE = 0.53), p = 0.033. In the multivariate model only positive margin was selected as the best predictor of relapse, OR = 5.01 (SE = 0.60), p = 0.007.ConclusionsThe simple clinical aspect of slow or fast PA progression is of great practical importance and can constitute a surrogate of the final histopathological information that is derived from the surgical specimen. The slow or fast nature of the PA to some extent indicates prognostic features such as recurrence risk. This finding requires correlation with histological and molecular features in further stages of research.

The result of the reaction of free radicals with biomolecules is the formation of substances with the potential of inducing oxidative damage, a condition known as oxidative stress. There are voluminous literature data reporting the association, both as a cause and as a consequence, between different diseases and oxidative stress. In this study, 144 female dogs with mammary neoplasia were analyzed. The animals were submitted to clinical evaluation for disease staging, hematological evaluation, serum biochemistry (renal and hepatic function tests), and dosage of the oxidative damage biomarker, malondialdehyde (MDA), at the time of its approach and 30 days after treatment. A control group of 100 healthy animals was also submitted to determination of serum MDA levels. The mean age of the animals affected by mammary neoplasms was 9.88±2.95 (4 to 14) years, while in healthy animals it was 2.31±1.90 years (1 to 6). Of the 144 animals, 113 (78.9%) had malignant neoplasms, and 15, 21, 46, 17 and 14 animals were in clinical stage I, II, III, IV and V respectively and the carcinoma in a mixed tumor was the most frequent histological pattern in this group (26%). Thirty-one animals were diagnosed with benign neoplasms and mammary adenoma was the most frequent histological pattern in 15 animals (51.61%). Hematological changes in the preoperative period were observed in 44 (38.9%) and 12 (38.7%) animals with malignant and benign neoplasias, respectively, and there was a positive correlation between anemia and higher levels of MDA (P=0.0008) for animals with malignant tumors. Regarding serum biochemical parameters, the most frequent alterations in animals with malignant neoplasms were elevated ALT levels in 12 animals (10.6%), creatinine in 10 animals (8.84%) and urea in eight animals (7.07%). Females with benign neoplasms presented less occurrence of changes in these parameters. In the group of healthy animals (control), the mean serum MDA values were 12.08±4.18, whereas in the pre-treatment group, mean MDA was 24.80±5.74 for bitches with benign neoplasms and 32.27±10.24 for bitches with malignant tumors. A significant increase (P

Background: The incidence of cancer has increased due to greater longevity of the animals as a consequence of better control of other diseases, improvement of nutrition and good practices in preventive medicine. Malignant tumors can cause paraneoplastic syndrome as hypercalcemia, anemia, cachexia, among others and consequent cardiovascular disorders. The electrocardiography (ECG) is a complementary exam that can reveal with its traces these rhythms disorders. Based on that, the objective of this study was to evaluate the ECG in bitches with mammary tumors in order to detect in which type of tumor would be found significant changes as in cardiac rhythm, ECG waves, segments or cardiac axis.Material, Methods & Results: Twenty-nine female dogs were used in this study, being 19 bitches with mammary tumor, and they were allocated in three groups: G1: control group (n=10), G2: benign neoplasia group (n=6) and G3: malignant neoplasia group (n=13). The most prevalent type of neoplasia in G2 was the benign mixed tumor (83%), followed by adenoma (17%) whereas in G3: carcinoma in mixed tumor (61%); papillary carcinoma (23%). Regarding cardiac rhythm, it was found sinus arrhythmia (SA) and normal sinus rhythm (NS): G1: 50% SA and 50% NS; G2: 67% SA and 33% NS; G3: 54% SA and 46% NS. No ventricular or atrial arrhythmias were detected. For other parameters in G1, G2 and G3, respectively (mean±Std error): FC (bpm): 110±9.2, 120±8.5, 124±7,5; P (ms): 48±1.6, 51±1.8, 50±1.2; P (mV) 0.19±0.02, 0,2±0.02, 0,19±0.02; PR (ms): 94±4.3, 93±5.5, 89±3.9; QRS (ms): 56±1.54, 60±4, 62±1.2; R (mV): 1.1±0.06, 1.2±0.24, 0.9±0.13; QT (ms): 203±9.4; 204±7.9; 182±15.6; and cardiac axis (°): 66±6.2, 61±7.9, 70±7.5. There were no significant differences for all cardiac parameters and also for ST interval and T wave morphology.Discussion: All electrocardiographic parameters found are in accordance with other studies carried out in the canine species. The results regarding tumor types differ from what was found in another study, in which in 18 bitches with mammary tumors, 55% were benign, with prevalence of adenomas (38%) followed by benign mixed tumors, and 45% malignant, with adenocarcinoma prevailing (22%). In another study, it was verified in 63 bitches the predominance of tubular carcinoma (26.56%) and carcinoma in mixed tumor (23.44%). Diverging from the electrocardiographic parameters of this study, a significant difference was found in the R wave amplitude value in the research by Barros et al., (2015) who performed computerized electrocardiography in 50 dogs, not only with mammary neoplasms (55% mammary carcinomas), but also in mastocytomas, lymphomas, benign tumors and other sarcomas. This author found out that the R wave amplitude values of the neoplasia group were lower when compared to the control group. In addition, we suspect that other types of tumors could result in more paraneoplastic syndrome than the mammary neoplasms found in this research. Neoplasms as lymphomas (T cells), apocrine gland anal sac adenocarcinomas, multiple myelomas and thymomas are known as a cause of hypercalcemia of malignancy and histiocytic sarcomas, myelomas, leukemia and lymphomas causing anemia. In conclusion, ECG has no changes in benign or malignant mammary tumors in dogs. However, this fact does not exclude the importance of its performance in pre-anesthetic evaluations.

Purpose: The aim of this study was to evaluate the clinical and pathological characteristics of mixed endometrial carcinoma (MEC).Materials and Methods: The clinical and pathological records of the 29 MEC patients, who were operated on and regularly followed up in the clinic between January 2000 and December 2019, were reviewed. Clinic-pathologic features and survival in the MEC group (n=29) were compared to pure serous (n=42) and pure clear cell adenocarcinomas (n=13). Clinical features, operation characteristics, pathological findings, myometrial invasion degree (MI), lymph node involvement (LNI), lymphovascular space invasion (LVSI), adjuvant therapies, and follow-up data of the patients and their effects on survival were investigated. Results: Eighteen of the cases had endometrioid + serous, 7 had endometrioid + clear, 3 had endometrioid + serous, and 1 had clear + serous histopathology. Laparoscopic surgery was performed in 8 of the cases (27.6%) in the mixed group. Stage, the rate of LVSI, LNI, MI ≥50%, and omental metastasis were similar among the groups. There were no significant differences in the rates of receiving adjuvant therapy among the groups. Overall survive (OS) was similar among the groups. Conclusion: MECs are tumors that can be difficult to diagnose and manage. There was no difference between MEC and pure serous carcinoma (SC) and pure clear cell carcinoma (CC) in terms of clinicopathological features and prognosis. In addition to histopathological features, revealing and evaluating their molecular properties will help us to better understand this group of tumors., Amaç: Bu çalışmada miks endometrial karsinomun (MEK) klinik ve patolojik özelliklerini değerlendirilmesi amaçlanmıştır.Gereç ve Yöntem: Ocak 2000 - Aralık 2019 tarihleri arasında kliniğimizde ameliyat edilen ve düzenli takip edilen 29 MEK hastasının klinik ve patolojik kayıtları gözden geçirildi. MEK grubundaki (n = 29) klinik-patolojik özellikler ve sağkalım, saf seröz (n = 42) ve saf berrak hücreli adenokarsinomlar (n = 13) ile karşılaştırıldı. Hastaların klinik bulguları, operasyon özellikleri, patolojik bulguları, miyometriyal invazyon derecesi (MI), lenf nodu tutulumu (LNI), lenfovasküler alan invazyonu (LVSI), adjuvan tedaviler ve takip verileri ve sağkalıma etkileri araştırıldı.Bulgular: Olguların 18'inde endometrioid + seröz, 7'sinde endometrioid + berrak hücreli, 3'ünde endometrioid + seröz ve 1'inde berrak hücreli+ seröz histopatoloji vardı. Hastaların ortalama yaşı 63,2 ± 12,1 yıldı. Mikst gruptaki olguların 8'ine (% 27,6) laparoskopik cerrahi uygulandı. Evre, LVSI, LNI, MI ≥% 50 ve omental metastaz oranları gruplar arasında benzerdi. Adjuvan tedavi alma oranları için gruplar arasında önemli bir fark yoktu. Genel olarak hayatta kalma süresi gruplar arasında benzerdi.Sonuç: MEK'ler, teşhis edilmesi ve yönetilmesi zor olabilen tümörlerdir. Klinikopatolojik özellikler ve prognoz açısından MEK ile saf seröz karsinom ve saf berrak hücreli karsinom arasında fark yoktu. Histopatolojik özelliklerinin yanı sıra moleküler özelliklerinin ortaya çıkarılması ve değerlendirilmesi bu grup tümörleri daha iyi anlamamıza yardımcı olacaktır.

Pleomorphic adenoma is a benign mixed tumor composed of epithelial and myoepithelial cells, less commonly occurring in minor salivary glands or in children. A case of juvenile pleomorphic adenoma missed clinically and incidentally found in cone beam CT images is described. Clinical, radiographic, and histologic images are presented.

Background A cutaneous ciliated cyst (CCC) is a rare, benign tumor in young female adults, which is usually found on the lower extremities. Case summary We found an uncommon location of CCC in the anterolateral cervical area and reviewed the literature. A 20-year-old female complained of a well-defined, painless, palpable mass that started several years ago. The mass was tense and movable and located at the anterolateral aspect of the neck. Imaging showed a non-enhancing round mass. Surgical excision biopsy was performed, and the cystic mass was revealed to be a CCC. Conclusion The rare location of CCC can be found in anterior neck area, which should be another diagnostic option for mass on anterior neck.

Here, we report the case of a 54‐year‐old man with a history of squamous cell carcinoma (SCC) of the lung, who developed bilateral neuroendocrine carcinoma (NEC) of the palatine tonsils. Faced with this atypical situation, another biopsy of the lung lesion was performed, revealing NEC histology patterns. This article describes the first case of metastasis to the bilateral palatine tonsils from the NEC component of a mixed NEC/SCC of the lung, highlighting the importance of reconsidering the diagnosis of the primary tumor histology, particularly in lung cancer with the possible presence of mixed tumor after phenotype transdifferentiation of the primary tumor. Key points Significant findings of the study Mixed lung carcinoma can be revealed after the presence of neuroendocrine carcinoma metastasis. What this study adds Bilateral neuroendocrine carcinoma of the palatine tonsils should be considered as metastases, particularly in the presence of lung cancer with a poor response to treatment., Mixed lung carcinoma can be revealed after the presence of neuroendocrine carcinoma metastasis.Bilateral neuroendocrine carcinoma of the palatine tonsils should be considered as metastases, particularly in the presence of lung cancer with a poor response to treatment.

An 8-year-old girl was admitted with four limb weakness for 2 months. Hyperactive reflexes were observed in all four limbs, and positive Hoffmann’s signs were revealed. An MRI spine with the coronal slide detected two tumors, first in the intradural and intramedullary space at the C2–C4 level and the second in the intradural and extramedullary space at the C5–C7 level. Axial T1W contrast MRI brain showed a tumor in the left parietal region. The patient underwent spine surgery first and following by brain surgery. No invasive lesions were remarked during surgery. Histological findings showed meningothelial meningioma and ependymoma from the spine and rhabdoid meningioma from the brain. Two months after the second surgery, the patient recovered fully with no symptoms and was able to participate in all regular activities in life. This work is the first report of a mixed tumor with distinct spinal meningioma and ependymoma components occurring in the cervical spinal cord at the C2–C7 level and coexisting with cranial meningioma. The remarkable result was that the patient fully recovered without any symptoms after receiving two surgeries.

Lacrimal gland neoplasms comprise up to 18% of all orbital masses clinically and histologically. Much of our current core knowledge regarding lacrimal gland tumors stems from prior study of their more common counterparts, the salivary glands. The prognosis for each lacrimal gland tumor is contingent upon proper clinical evaluation and ultimately the histopathologic diagnosis. We describe a case of an invasive carcinoma ex-pleomorphic adenoma (Ca-ex-PA) with a cystadenocarcinoma component arising from the lacrimal gland in the absence of any previously diagnosed pleomorphic adenoma (benign mixed tumor) or prior incisional surgery. This case illustrates the importance of the histopathologic assessment including immunohistochemistry and genetic testing to narrow a differential diagnosis and potentially aid or guide therapy in the future. Our finding suggests that carcinoma of the lacrimal gland may be derived from previously undiagnosed and perhaps even subclinical pleomorphic adenoma.

OBJECTIVE: To describe the clinical and sonographic characteristics of malignant ovarian yolk sac tumors (YSTs). METHODS: In this retrospective multicenter study, we included 21 patients with a histological diagnosis of ovarian YST and available transvaginal ultrasound images and/or videoclips and/or a detailed ultrasound report. Ten patients identified from the International Ovarian Tumor Analysis (IOTA) studies had undergone a standardized preoperative ultrasound examination, by an experienced ultrasound examiner, between 1999 and 2016. A further 11 patients were identified through medical files, for whom ultrasound images were retrieved from local image workstations and picture archiving and communication systems. All tumors were described using IOTA terminology. The collected ultrasound images and videoclips were used by two observers for additional characterization of the tumors. RESULTS: All cases were pure YSTs, except for one that was a mixed tumor (80% YST and 20% embryonal carcinoma). Median age at diagnosis was 25 (interquartile range (IQR), 19.5-30.5) years. Seventy-six percent (16/21) of women had an International Federation of Gynecology and Obstetrics (FIGO) Stage I-II tumor at diagnosis. Fifty-eight percent (11/19) of women felt pain during the ultrasound examination and one presented with ovarian torsion. Median serum α-fetoprotein (S-AFP) level was 4755 (IQR, 1071-25 303) µg/L and median serum CA 125 level was 126 (IQR, 35-227) kU/L. On ultrasound assessment, 95% (20/21) of tumors were unilateral. The median maximum tumor diameter was 157 (IQR, 107-181) mm and the largest solid component was 110 (IQR, 66-159) mm. Tumors were classified as either multilocular-solid (10/21; 48%) or solid (11/21; 52%). Papillary projections were found in 10% (2/21) of cases. Most (20/21; 95%) tumors were well vascularized (color score, 3-4) and none had acoustic shadowing. Malignancy was suspected in all cases, except in the patient with ovarian torsion, who presented a tumor with a color score of 1, which was classified as probably benign. Image and videoclip quality was considered as adequate in 18/21 cases. On review of the images and videoclips, we found that all tumors contained both solid components and cystic spaces, and that 89% (16/18) had irregular, still fine-textured and slightly hyperechoic solid tissue, giving them a characteristic appearance. CONCLUSION: Malignant ovarian YSTs are often detected at an early stage, in young women usually in the second or third decade of life, presenting with pain and markedly elevated S-AFP. On ultrasound, malignant ovarian YSTs are mostly unilateral, large and multilocular-solid or solid, with fine-textured slightly hyperechoic solid tissue and rich vascularization. © 2020 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of the International Society of Ultrasound in Obstetrics and Gynecology.. ispartof: ULTRASOUND IN OBSTETRICS & GYNECOLOGY vol:56 issue:2 pages:276-284 ispartof: location:England status: published

Adrenal corticomedullary mixed tumors (CMMTs) are extremely rare; with only 20 cases being reported to date, the pathogenesis has remained elusive. A 31-year-old woman developed gestational hypertension with psychiatric disturbances persistent to postpartum and was diagnosed with pheochromocytoma, for which adrenalectomy was performed. Histological findings showed mixed adrenocortical adenoma and pheochromocytoma. Double immunostaining of inhibin and INSM1 (insulinoma-associated protein 1) showed that the 2 tumor components had distinct functional properties. Exome analysis of peripheral leukocytes and tumor (singular, as anatomically it is only 1 mass) revealed a homozygous germline FGFR4-G388R variant. As a readout of the variant, serine phosphorylation of signal transducer and activator of transcription 3 (STAT3) was detected only in the nucleus of adrenocortical adenoma component but not in the pheochromocytoma component. No tyrosine phosphorylation of STAT3 was detected. We report a case of CMMT with the germline FGFR4-G388R variant. Although additional studies are required, our immunohistochemical analysis suggests that the variant may play a role in the development of the adrenocortical component within the pheochromocytoma, leading to CMMT.

Background. Hemangioblastoma occurs mainly in the cerebellum and rarely in the cerebrum. Objective. The present study aimed to analyze the clinical manifestations and radiological and pathological features of cerebral hemangioblastoma, and to improve the recognition of this tumor and avoid misdiagnosis. Methods. The characteristics of 6 patients with cerebral hemangioblastoma were analyzed, and a retrospective review of cerebral hemangioblastoma reported in the literature was performed. Results. All 6 patients were female, aged from 22 to 70 years (55 years on average), and all cases were wild-type sporadic, in which 4 cases occurred in the frontal lobe and 2 cases occurred in the parietal lobe. Imaging revealed a solid tumor in 4 cases, a cystic tumor in 1 case, and a mixed tumor in 1 case. Microscopically, the morphology and immunophenotype of tumor cells were not different from those of classical hemangioblastoma. All 6 patients survived tumor free during the follow-up period. Conclusions. Cerebral hemangioblastoma often simulates the imaging characteristics of meningioma or glioma. Enough attention should be paid to differential diagnosis before the operation, and exact diagnosis relies on the pathological examination.

Objective To describe a family in which 3 members presented with mixed epithelial tumor of the kidney (MEST) and were found to possess a germline mutation in CDC73, a gene which is associated with hyperparathyroidism-jaw tumor syndrome (HPT-JT). Materials and Methods Blood and tumor DNA from three family members who presented with a primary diagnosis of MEST was subjected to targeted gene sequencing to identify potential genetic components. Results A germline start codon mutation (p.M1I) in CDC73 was identified in all 3 family members who presented with MEST and 2 tumors from 1 patient demonstrated somatic copy-neutral loss of heterozygosity. Patients presented with no evidence of hyperparathyroidism or jaw tumors, but both female patients had hysterectomies at an early age due to excessive bleeding and numerous fibroids, which is common in HPT-JT. A germline p.M1I mutation has been previously reported in a family with clinical features of HPT-JT. Conclusion Patients with MEST may be at risk for HPT-JT and CDC73 germline mutation testing of MEST patients should be considered.