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An intracranial collision tumor is a rare lesion composed of two histologically different neoplasms in the same anatomic location. Even more rare is the collision tumor of a solitary fibrous tumor/hemangiopericytoma (SFT/HPC) and meningioma. The patient was a 46-year-old woman who had a 40 × 35 × 30-mm mass in the vermis of the cerebellum. Histologically, the mass consisted of two different components. One component showed the morphology of meningioma (World Health Organization (WHO) grade I), and the other component exhibited small round cell proliferation with hypercellular density, which was revealed to be SFT/HPC (WHO grade III) based on STAT6 immunohistochemistry. STAT6 showed completely different immunohistochemistry results in these two components (nuclear-negative in meningioma and nuclear-positive in SFT/HPC). Since these two neoplasms are associated with different prognoses, they should be distinguished from each other. When meningioma and an SFT/HPC-like lesion are identified morphologically, it is important to recognize the presence of such a collision tumor composed of meningioma and SFT/HPC, and identify the SFT/HPC component by employing STAT6 immunohistochemistry.

Aim This study aimed to evaluate the clinical features and pregnancy outcomes of placental mesenchymal dysplasia (PMD) in Japan. Methods We requested detailed clinical information and placental tissue of PMD cases in 2000-2018 from Japanese facilities with departments of obstetrics and gynecology and analyzed the pregnancy course and neonatal outcomes. Results We collected 49 cases of PMD. Of 18 patients with measured maternal serum alpha-fetoprotein (MSAFP) levels, 15 (83.3%) had elevated levels. Maternal serum human chorionic gonadotropin (MShCG) levels were transiently elevated in five (17.8%) of 28 patients. Forty-seven patients continued their pregnancies. All pregnancies were singleton and 40 (85.1%) were associated with adverse events including fetal growth restriction (FGR), threatened premature delivery, fetal demise, and hypertensive disorder of pregnancy in 34 (72.3%), 14 (29.8%), eight (17.0%), and six (12.8%) patients, respectively. Of 47 infants, there were eight stillbirths. There were 40 (85.1%) female infants, and eight (17.0%) had Beckwith-Wiedemann syndrome. Of 39 live births, 23 (59.0%) were associated with premature induction of labor or cesarean section for obstetric indications related to FGR. Eighteen (46.2%) neonates had complications. PMD-affected placentas were pathologically heterogeneous in both grossly PMD-affected and non-affected areas. Conclusions Our study included the largest number of PMD cases with detailed clinical information. PMD is a high-risk condition for both the mother and the child. Elevated MSAFP levels with normal MShCG levels indicate PMD. Conventional perinatal management of FGR in Japan might be effective in reducing the fetal mortality rate.

Endometrial stromal sarcoma (ESS) is a rare uterine malignancy that requires accurate pathological diagnosis for proper treatment. This study aimed to clarify the discrepancies in the pathological diagnosis of ESS and obtain practical clues to improve diagnostic accuracy. Between 2002 and 2015, 148 patients with low-grade ESS (LGESS), high-grade ESS (HGESS), undifferentiated endometrial sarcoma (UES), or undifferentiated uterine sarcoma (UUS) diagnosed at 31 institutions were included. We performed immunohistochemistry, real-time polymerase chain reaction for JAZF1-SUZ12 and YWHAE-NUTM2A/B, and break-apart fluorescent in situ hybridization for JAZF1, PHF1, and YWHAE. Central pathology review (CPR) was performed by six pathologists. After CPR, LGESS, HGESS, UES/UUS, and other diagnoses were confirmed in 72, 25, 16, and 31 cases, respectively. Diagnostic discrepancies were observed in 19.6% (18/92) of LGESS and 34% (18/53) of HGESS or UUS/UES. Adenosarcomas, endometrial carcinomas, carcinosarcomas, and leiomyosarcomas were common diagnostic pitfalls. JAZF1-SUZ12 transcript, PHF1 split signal, and YWHAE-NUTM2A/B transcript were mutually exclusively detected in 23 LGESS, 3 LGESS, and 1 LGESS plus 3 HGESS, respectively. JAZF1-SUZ12 and YWHAE-NUTM2A/B transcripts were detected only in cases with CPR diagnosis of LGESS or HGESS. The CPR diagnosis of LGESS, HGESS, and UUS was a significant prognosticator, and patients with LGESS depicted a favorable prognosis, while those with UUS showed the worst prognosis. Pathological diagnosis of ESS is often challenging and certain tumors should be carefully considered. The accurate pathological diagnosis with the aid of molecular testing is essential for prognostic prediction and treatment selection.

To analyze the clinical behavior of neuroendocrine tumors (NETs) of the uterine cervix, we conducted a multicenter, retrospective study of 193 patients.We evaluated the prognosis of NETs according to the new International Federation of Gynecology and Obstetrics (FIGO) staging system, compared the clinical response to different chemotherapy regimens, and compared different histological subtypes of NETS.Diagnoses of the subjects were atypical carcinoid tumor (ACT, n = 37), small cell neuroendocrine carcinoma (SCNEC, n = 126), large cell neuroendocrine carcinoma (LCNEC, n = 22), and NET, not elsewhere classified (n = 8), according to central pathological review. According to FIGO 2018, 69, 17, 74, and 33 patients were at stage I, II, III, or IV, respectively. Five-year survival was 64.5%, 50.1%, 30.2%, and 3.4% for patients at stage I, II, III and IV. About 40% of patients with stage IIIC1 survived5 years. On multivariate analyses, locally-advanced disease, para-aortic node metastasis, distant metastasis, and4 cycles of chemotherapy were associated with poor survival. Histological subtype and pelvic node metastasis had no prognostic significance. Response rates to etoposide-platinum (EP) or irinotecan-platinum (CPT-P) regimens were 43.8% (28/64), but only 12.9% to a taxane-platinum (TC) regimen (4/31). The response rate for ACT was 8.7% (2/23), significantly less than the 36.6% for high-grade neuroendocrine carcinomas (HGNEC: both SCNEC and LCNEC, 41/111).Locally-advanced, extra-pelvic disease and insufficient chemotherapy were independent prognostic factors for cervical NET. HGNEC showed good responses to EP or CPT-P but not TC. Chemotherapy was less effective for ACT, which had a prognosis identical to HGNEC.

Intraosseous lipoma (IOL) is a benign bone tumor that usually arises from the lower limb and rarely arises from the skull. Radiological diagnosis of a typical case is not problematic due to its characteristic calcification and marginal sclerosis. Here, we report a case of calvarial IOL in the early stage lacking conventional radiopathological features. The patient is a 7-year-old girl who presented with a slow-growing protuberance on the vertex of the head. Computed tomography displayed a low-density mass without calcification that was continuous with the surrounding diploe. The mass was resected piece by piece for diagnostic and cosmetic reasons. Histologically, the specimen consisted of bony trabeculae and intertrabecular adipose tissue, which resembled normal fatty marrow. However, adipose tissue was considered neoplastic since it lacked hematopoietic elements. The final diagnosis of IOL was made by radiopathological correlation. This case suggests that IOL should be included in the differential diagnosis of diploic expansion, even if calcification is absent. The histology of an early-stage IOL resembles normal fatty marrow, but recognizing the absence of hematopoietic elements aids the diagnosis. Also, our literature review indicates that such cases are likely to be encountered in the calvaria than cranial base.

Dysgerminoma is a malignant ovarian germ cell tumor, and unlike sex-cord stromal tumors, endocrine manifestation is considered rare. Here, we report the first case of dysgerminoma presenting precocious puberty. The patient is a 7-year-old girl who presented with a breast development in Tanner stage 3. Serum estradiol (E2) was markedly elevated while luteinizing hormone (LH) and follicle-stimulating hormone (FSH) were suppressed below the detection limit. Microscopically, the right ovarian mass displayed nests of large polygonal cells and fibrous septa which were focally concentrated by theca-like plump spindle cells. Immunohistochemistry revealed that the spindle cells expressed various steroidogenic enzymes involved in estrogen biosynthesis including P450 aromatase. The tumor was diagnosed with pure dysgerminoma with estrogen-producing functioning stroma. After the operation, serum E2 declined below the detection limit; LH and FSH returned within the normal range. This case demonstrates that even a conventional dysgerminoma can present endocrine manifestation through functioning stroma.

Purpose We aimed to determine appropriate treatment guidelines for patients with stages I–II high-grade neuroendocrine carcinomas (HGNEC) of the uterine cervix in a multicenter retrospective study. Patients and methods We reviewed the clinicopathological features and prognoses of 93 patients with HGNEC of International Federation of Gynecology and Obstetrics (FIGO) stages I and II. All patients were diagnosed with HGNEC by central pathological review. Results The median overall survival (OS) and disease-free survival (DFS) were 111.3months and 47.4months, respectively. Eighty-eight patients underwent radical surgery, and five had definitive radiotherapy. The hazard ratio (HR) for death after definitive radiotherapy to death after radical surgery was 4.74 (95% confidence interval [CI], 1.01–15.90). Of the surgery group, 18 received neoadjuvant chemotherapy. Pathological prognostic factors and optimal adjuvant therapies were evaluated for the 70 patients. Forty-one patients received adjuvant chemotherapy with etoposide–platinum (EP) or irinotecan–platinum (CPT–P). Multivariate analyses identified the invasion of lymphovascular spaces as a significant prognostic factor for both OS and DFS. Pelvic lymph node metastasis was also a prognostic factor for DFS. Adjuvant chemotherapy with an EP or CPT–P regimen appeared to improve DFS (HR=0.27, 95% CI, 0.10–0.69). A trend toward improved OS was also observed, but was not statistically significant (HR=0.39, 95% CI, 0.15–1.01). Conclusion Radical surgery followed by adjuvant chemotherapy with an EP or CPT–P regimen was optimal treatment for stages I and II HGNEC of the uterine cervix.

A 50-year-old man presented with dizziness and hearing disturbance in the right ear. Magnetic resonance imaging (MRI) revealed a well-enhanced mass lesion in the right cerebellopontine (CP) angle that appeared to originate in the cerebellum. A surgical specimen obtained at the subtotal resection with craniotomy revealed a diffuse large B-cell lymphoma (DLBCL). During the three courses of chemotherapy with high-dose methotrexate (MTX) with leucovorin rescue, he developed a right abducens palsy, left oculomotor palsy, left facial palsy, right trigeminal sensory disturbance, and paraparesis. Although the brain MRI showed that the CP angle tumor had disappeared completely following chemotherapy, enhanced lesions along the cauda equina were detected on a lumbar spine MRI. FDG-PET (18 F-fluorodeoxyglucose positron emission tomography) revealed multiple high-uptake abnormalities in the cranial nerves and spinal nerves. Tumor cells were found in the cerebrospinal fluid specimen from a lumbar puncture. Craniospinal irradiation was performed, including all the abnormal FDG high-uptake areas, and was effective in relieving the patient's symptoms. On FDG-PET, the high-uptake abnormalities in the peripheral nerves disappeared. However, five weeks after the irradiation, he developed right trigeminal sensory disturbance, hoarseness, dysphagia, and right arm pain. FDG-PET disclosed multiple high-uptake abnormalities in more peripheral portions of the cranial nerves and spinal nerves. Chemotherapy with rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine (Oncovin®), and prednisolone (R-CHOP) was then resorted to which mitigated his symptoms. On follow-up FDG-PET, the high-uptake abnormalities in the peripheral nerves disappeared again.

Ovarian high-grade serous adenocarcinoma responds well to regular platinum/taxane chemotherapy, while borderline tumor survives and demonstrates persistent disease. A 69-year-old Japanese woman was suspected for having advanced ovarian carcinoma. MRI showed cystic tumor containing solid component of the right adnexal region with massive ascites and peritoneal dissemination. Serum CA125 was elevated to 203 µ/ml; however, no remote metastases were detected. Laparotomy revealed that peritoneal carcinomatosis spreads out to omentum and subphrenic area. Omentum was partially removed with big tumor nodules that histologically demonstrated the high-grade serous adenocarcinoma with positive ascites cytology. After 6 cycles of postoperative chemotherapy with docetaxel and carboplatin, she received second surgery where the known residual bilateral adnexa and all of the persistent tumors were perfectly resected. Pathological examination of the tumor revealed serous borderline tumor with microinvasion and no evidence of residual high-grade serous carcinoma with negative ascites cytology. This is the extremely rare case of concurrent high-grade serous carcinoma and borderline tumor demonstrating differential chemo-sensitivity.

Subtypes of stx1 and stx2 in 45 Shiga toxin-producing Escherichia coli (STEC) strains isolated from cattle were investigated by PCR. Only subtype stx1a was detected among all the stx1-positive strains. The major stx2 subtype was stx2a followed by stx2d, stx2c, stx2b, and stx2g in decreasing order of frequency. stx2c was found in strains of serotypes O157 and O174. stx2d was found in 11 strains. These strains were confirmed by DNA sequencing to carry both the activatable tail and the END motif; all were eae-negative, and 3 contained stx2d as the only stx. stx2g was found in 2 strains in association with stx2a, estA1, and astA. In addition, 7 hybrid strains of shigatoxigenic and enterotoxigenic E. coli (STEC/ETEC) were found to harbor one or both of stx1a and stx2a (stx1a/stx2a) and estA1. Among 27 serotypes of STEC strains isolated from cattle, O157:H7 and O109:H- strains were eae-positive. Other putative adhesin genes, such as saa, iha, espP, and lpfAO113 were detected in more than 12 serotypes.

Struma ovarii exhibiting malignant histology are uncommon, and aggressive clinical courses with initial extraovarian spread are even more rare. This report describes a case of malignant struma ovarii with a predominant anaplastic carcinoma component. A 65-yr-old, gravida 2, para 2, female presented with lower abdominal discomfort and pain. She had a 12×10×7.5 cm tumor in the right ovary. Intraoperative diagnosis was high-grade spindle cell tumor. Right salpingo-oophorectomy and hysterectomy were performed. Macroscopically, the tumor invading the right tube was a yellow-white solid mass with focal microcysts containing greenish liquid and focal calcification. The tumor was histologically characterized by a spindle cell and pleomorphic sarcomatous component, and a minor component of benign-looking thyroid tissue with ossification. Immunohistochemically, the sarcomatous component was focally positive for CAM 5.2, EMA, thyroid transcription factor-1, and thyroglobulin, indicating anaplastic carcinoma. The patient was treated with chemotherapy and is alive, yet with tumor, 25 mo after surgery. This is the first case of malignant struma ovarii with a predominant component of anaplastic carcinoma. This type of malignant struma ovarii may lead to diagnostic problems, and sampling and differential diagnosis among sarcomatous ovarian tumors are important for making the correct diagnoses.

Pathogenic genes such as stx1, stx2, STh gene, STp gene, LT gene, invE, eae, aggR, afaD, astA, cdt and cnf were investigated in Escherichia coli isolated from cattle during Nov. 2012 and Aug. 2013. Plural pathogenic genes were concurrently detected by multiplex PCR, and screen-positive genes were confirmed and sub-classified by PCR. Among 100 cattle investigated, 180 E. coli strains with diarrheic genes (DEC) were detected in 79 cattle, and 45 of them, isolated from 32 cattle, were Shiga toxin-producing E. coli (STEC). More than 30% of cattle carried astA, cdt, cnf and stx2 in descending order. STh gene, LT gene, invE, aggR and afaD were not detected in this study. Both stx1 and stx2 were concurrently detected from 6 of 45 STEC strains and stx2 alone was detected from 19. Seventeen STEC strains carried STp gene, astA, or cdt along with stx1 or stx2. Additionally, 135 remaining DEC were classified into 18 enterotoxigenic E. coli with STp gene, 25 enteropathogenic E. coli with eae, and 92 other DEC with astA, cdt and cnf. Both O and H serotypes were identified in 48 strains, including O157 : H7, O1H7 and so on. O157 : H7 were identified in 3 strains that carried stx2 and eae together, as found in human pathogenic strains isolated from patients with gastroenteritis and hemolytic-uremic syndrome.

This case report describes a tubulosquamous polyp resembling a penis in the uterine cervix. A 34-yr-old, gravida 0, para 0, woman showed an 18 × 8 × 5 mm polypoid lesion in the uterine ectocervix. The polyp had a penis-like appearance; the tip looked like glans penis and the middle portion resembled the shaft of the penis. Its surface was covered by squamous epithelium, and tissues resembling those of a urethra, corpus spongiosum penis, and external orifice urethra were observed. Foreskin-like tissues were also observed, although a corpus cavernosum penis was not seen. Skene glands and Cowper glands were also observed. Immunohistochemically, Skene glands and the urethra-like epithelium were focally positive for prostate-specific antigen and/or prostatic acid proteins. Histologically and immunohistochemically, the polypoid lesion overlapped with a tubulosquamous polyp of the vagina and ectopic prostatic tissue of the uterine cervix and encompassed these lesions in the lower female genital tract. The most likely theory of histogenesis is a developmental anomaly and misplacement of Skene glands.

We identified the stepwise increase of MIB-1 index in a long-surviving malignant peritoneal mesothelioma (MPM) patient with a history of frequent relapse. A 29-year-old Japanese woman showed upper abdominal induration with adnexal tumor. Imaging study with biochemical analyses strongly suggested peritoneal tumor. On primary surgery, all tumors were resected completely without any residual tumor. Histologically, the tumor was diagnosed as MPM, for which she received adjuvant chemotherapy containing platinum agent. Two years later, the tumor relapsed in her pelvic cavity, but was resected completely with hysterectomy and salpingo-oophorectomy. Histologically, the tumor was diagnosed as MPM relapse. She underwent intraperitoneal chemotherapy with cisplatin that achieved progression-free survival of 5 years. However, relapse was detected again in pelvic cavity without any dissemination in upper abdominal cavity. The tumors were completely removed and were revealed to be compatible with MPM. She received gemcitabine and carboplatin chemotherapy. However, 2 years later, the tumor relapsed again in left upper abdominal cavity, for which she wouldn’t receive 4th treatment. To investigate the longevity of this patient in association with the histologic findings, the MIB-1 index was examined in the primary and relapse tumors. The rate of MIB-1 index positive cells was calculated by counting 500 cells. MIB-1 indices were 4.2 ± 1.1 (mean ± SE), 11.8 ± 2.3, and 37.3 ± 2.5 in primary, 1st- and 2nd-relapsed tumor, respectively, demonstrating stepwise increase of MIB-1 expression over the surviving time of more than 9 years. Increase in MIB-1 index was not associated with mitotic index but may be indicating drug sensitivity, resulting in >2-year progression-free interval in each relapse.

A 65-year-old man was referred to our department due to repeated episodes of cholangitis in the past five years. Endoscopic retrograde cholangiopancreatography was performed, and a stricture of the lower bile duct was detected. At a later date, an irregular mucosa of the bile duct was confirmed using nasal endoscopy. Based on the biopsy results, the patient was diagnosed with bile duct cancer and subsequently underwent surgery. Postoperative histopathology did not show lymph node metastasis, and the condition was determined to be early-stage bile duct cancer. In the present case, it was presumed that the cancer had developed due to chronic cholangitis. Therefore, in patients with repeated episodes of cholangitis, attention should be focused on the possible and concomitant development of cancer.

Non-epithelial ovarian tumors have posed pathologic diagnosis and management challenges. The World Health Organization classification of Tumors of Female Reproductive Organs was revised in 2014, and the new version addresses several new concepts and the histological classification of non-epithelial ovarian tumors and tumor-like lesions that were not previously included. In the new WHO classification, sex cord-stromal tumors is divided into three categories, pure stromal tumor, pure sex cord tumor, and mixed sex-cord tumor.

Background Uterine leiomyomas are common uterine tumors, and typical cases of leiomyoma are easily diagnosed by imaging study. However, uterine leiomyomas are often altered by degenerative changes, which can cause difficulty and confusion in their clinical diagnosis. We describe the 17th reported case of a uterine leiomyoma clinically diagnosed as an ovarian tumor; however, the present case shows the most detailed radiological evaluation, including contrast-enhanced magnetic resonance imaging. We first show that a uterine leiomyoma can histologically mimic an adenomatoid tumor. Case presentation A 47-year-old premenopausal, nulliparous Japanese woman with a history of type 2 diabetes mellitus, hypertension, and hyperlipidemia had lower abdominal pain. Ultrasonography confirmed a 6-cm mass in the right-sided space of the pelvic cavity. Magnetic resonance imaging evaluation showed that a multilocular mass was present near the uterus, and a mucinous ovarian tumor was considered. Emergency surgery due to acute abdomen was performed under the diagnosis of pedicle torsion of the ovarian tumor. During surgery, a pedunculated uterine mass without stalk torsion was seen. The mass grossly contained serous and hemorrhagic fluids in the cavities, and pathology examination confirmed that the mass was a leiomyoma with hydropic and cystic degeneration. Anastomosing thin cord-like arrangements of the leiomyoma cells mimicked the architecture of adenomatoid tumors. The tumor cells were positive for the microphthalmia transcription factor but negative for other melanoma markers. Three days postoperatively, she was discharged without sequelae. Conclusions Marked intratumoral deposition of fluids may induce the multilocular morphology of a tumor, and the cellular arrangement of the tumor cells with hydropic degeneration mimicked an adenomatoid tumor in this case. Clinicians need to be aware that a subserosal leiomyoma with cystic and hydropic degeneration can mimic an ovarian tumor, and pathologists should be aware that such leiomyomas can mimic adenomatoid tumors. Additionally, perivascular epithelioid cell tumors should not be diagnosed only based on its immunoreactivity for the microphthalmia transcription factor.

Subtypes of stx1 and stx2 in 45 Shiga toxin-producing Escherichia coli (STEC) strains isolated from cattle were investigated by PCR. Only subtype stx1a was detected among all the stx1-positive strains. The major stx2 subtype was stx2a followed by stx2d, stx2c, stx2b, and stx2g in decreasing order of frequency. stx2c was found in strains of serotypes O157 and O174. stx2d was found in 11 strains. These strains were confirmed by DNA sequencing to carry both the activatable tail and the END motif; all were eae-negative, and 3 contained stx2d as the only stx. stx2g was found in 2 strains in association with stx2a, estA1, and astA. In addition, 7 hybrid strains of shigatoxigenic and enterotoxigenic E. coli (STEC/ETEC) were found to harbor one or both of stx1a and stx2a (stx1a/stx2a) and estA1. Among 27 serotypes of STEC strains isolated from cattle, O157:H7 and O109:H- strains were eae-positive. Other putative adhesin genes, such as saa, iha, espP, and lpfA

Female adnexal tumors of probable Wolffian origin (FATWOs) are rare neoplasms that originate from remnants of the Wolffian duct. These tumors often appear in the broad ligament and para-ovarian area where such remnants exist. FATWOs have specific pathological characteristics similar to those of remnants of the Wolffian duct. Since these tumors neither have any specific tumor markers nor specific features on scanning images, there has been no case report concerning the preoperative diagnosis of FATWOs. Diagnostic imaging technology has become remarkably developed nowadays, enabling physicians to make preoperative diagnosis more precisely. We report here a case of FATWO with detailed discussion of its MR images.

Autoimmune pancreatitis (AIP) can be difficult to distinguish from pancreatic cancer. We report a case of histopathologically proven AIP mimicking neuroendocrine tumor (NET) or pancreatic cancer in a 53-year-old man. He was referred to our hospital for further evaluation of a pancreatic mass detected on ultrasonography at a medical check-up. Abdominal ultrasonography showed a 15-mm hypoechoic mass located in the pancreatic body. Computed tomography revealed a tumor without any contrast enhancement, and magnetic resonance imaging demonstrated the mass to be hyperintense on diffusion-weighted image. Endoscopic retrograde cholangiopancreatography revealed slight dilatation of a branch of the pancreatic duct without stricture of the main pancreatic duct. The common bile duct seemed intact. Under suspicion of a non-functioning NET or malignant neoplasm, laparotomy was performed. At laparotomy, an elastic firm and well-circumscribed mass was found suggestive of a non-functioning NET, thus enucleation was performed. Histopathologically, the lesion corresponded to AIP.

Herein is presented a very rare case of alveolar rhabdomyosarcoma in the uterine corpus of a 72-year-old woman. The wall of the uterine corpus was replaced by multiple whitish-yellow, friable nodules, measuring up to 6 cm. Microscopically, the tumor was predominantly composed of round to polygonal cells arranged in an alveolar, papillary or nest pattern intermingled with multinuclear giant cells with abundant eosinophilic cytoplasm. Extensive sampling failed to show epithelial elements. Immunohistochemically, the tumor was positive for striated muscle markers such as myoglobin, myoD1 and myogenin. Metastatic lesions were found in the retroperitoneum and pelvic lymph nodes. The patient was treated by postoperative chemotherapy, but she died of systemic metastases 12 months after surgery.

A clinicopathologic and DNA flow cytometric study was performed on seven patients (three males, four females) with atypical adenoma of the thyroid gland, using formalin-fixed paraffin-embedded tissues. The results were compared with those of 30 follicular adenomas and 13 follicular carcinomas. The patients ranged in age from 32 to 74 years (mean: 55.8 years), and the mean follow-up period was 11.0 years. All patients except two who died of other diseases were free of thyroid disease after initial surgery. It showed that there was no evidence of clinical cancer in this follow-up study of patients operated on for atypical adenomas. Four of the atypical adenomas were diploid, two were aneuploid, and one was tetraploid. Twenty-seven of the 30 follicular adenomas were diploid. Three patients with aneuploid follicular adenoma were free of disease. Of the 13 follicular carcinomas with a mean follow up period of 6.9 years, five were diploid, seven were aneuploid, and one was tetraploid. Two patients with aneuploid follicular carcinoma and one with diploid carcinoma developed lung metastases, and one patient each with diploid and aneuploid follicular carcinoma died of disease. There was no significant correlation between histologic features, ploidy status and prognosis among follicular carcinomas. The results of this study suggest that DNA flow cytometric analysis is not a useful tool for predicting the clinical behavior of follicular neoplasms. Acta Pathol Jpn 42: 632–638, 1992.

Immunohistochemical, ultrastructural, and flow cytometric studies were performed on an ossifying soft-tissue tumor, presumed to be a variant of ossifying fibromyxoid tumor of soft parts, which was located in the subcutis of the left buttock of a 76 year old Japanese woman. Histologically, this was a benign-looking spindle, oval, or round cell lesion, having a fibrous capsule with a discontinuous rim of bone as seen in typical cases. However, this lesion was also characterized by a high degree of cellular proliferation in storiform and whorl arrangements, extensive ossification, osteoid and metaplastic bone formation and absence of myxoid features. In an immunohistochemical study using formalin-fixed, paraffin-embedded sections, many tumor cells expressed vimentin, S-100 protein, Leu-7, neuron specific enolase, and desmin. Ultrastructurally, this neoplasm consisted of fibroblast-like cells and myofibroblast-like cells. This tumor had an aneuploid DNA content. No recurrence has been observed for 16 months. These results suggest that the neoplastic cells may show the phenotypic expressions of myofibroblast and also osteogenic differentiation.

This study aimed to document chronologic histologic changes of endometrial biopsies from patients with endometrial adenocarcinoma on high-dose progestin therapy. Seven patients with presumptive FIGO stage IA endometrial adenocarcinoma treated with medroxyprogesterone acetate 600 mg/day were investigated retrospectively. Good response was defined as complete disappearance of carcinoma foci within 16 weeks of treatment and poor response as the presence of residual foci at 16 weeks. Two patients were poor responders and were excluded from the study, while five good responders were analyzed. Hematoxylin and eosin (H&E)–stained slides were reviewed and analyzed based on nine histologic features to describe the histology observed commonly in good responders. All the five good responders showed relatively uniform morphologic changes during the high-dose progestin therapy and the common histology was described as follows. The first change was swelling of the neoplastic glandular epithelial cells with pale vacuolated cytoplasm and round to oval nuclei. Mitotic arrest was also observed. Next, the epithelia were disrupted by lymphoplasmocytic infiltration and replaced by low cuboidal epithelium with or without squamous or morular metaplasia. The stromal area increased with predecidual changes. The final morphology was small atrophic glands scattered in predecidual stroma with dilated vessels. Therefore, the morphologic change of the endometrial biopsy observed in earlier stage of treatment might be able to predict good response to high-dose progestin therapy.

A 79-year-old man noticed nasal obstruction and bleeding caused by a pedunculated tumor in the left nasal cavity. The tumor revealed a malignant neoplasm predominantly composed of immature neuroepithelial cells with rosettes and palisading patterns, rhabdomyoblastic cells with positivity for desmin and cross-striations, and immature mucin-producing glandular cells. Small amounts of mature chondroid tissue and neurofibrillary matrix were also present. Therefore, the tumor was diagnosed as nasal teratocarcinosarcoma.

We report on a 61-year-old Japanese male with a pedunculated tumor in the common bile duct. The tumor consisted of two types of neoplastic cells. The majority showed atypical spindle- and giant-shaped features and proliferated densely in an inflammatory stroma, revealing a sarcomatous pattern. They expressed vimentin, KL-1, and CAM5.2. The remaining minority showed glandular and tubular features, occupied only less than 5%, located only in the tumor surface, and expressed wide spectrum keratin, KL-1, CAM5.2, epithelial membrane antigen, AE1/AE3, and carcinoembryonic antigen. CD68-positive osteoclast-like giant cells were also observed. Therefore, the patient was diagnosed as having an undifferentiated carcinoma, spindle and giant cell type.

Background The ovarian cellular fibrous tumor with mitotic figure >4 per 10 high power field without moderate to severe atypia is defined as mitotically active cellular fibroma according to the 2014 World Health Organization classification. As this category is new and rare now, we described here a case of MACF and reviewed the literature. Case We present a case of mitotically active cellular fibroma of the ovary with 10-year history that was treated with laparoscopic surgery. Methods We reviewed the relevant literature using PubMed search system and analyzed the previous cases. Results To date, only 5 cases of mitotically active cellular fibroma have been reported. Our patient is the first case of mitotically active cellular fibroma of the ovary treated with laparoscopic surgery. Conclusion MACF of the ovary is a newly defined category and few cases have been reported, while prognostic factors have also not yet been fully characterized. Long-term clinical follow-up is necessary.

Pathogenic genes such as stx1, stx2, STh gene, STp gene, LT gene, invE, eae, aggR, afaD, astA, cdt and cnf were investigated in Escherichia coli isolated from cattle during Nov. 2012 and Aug. 2013. Plural pathogenic genes were concurrently detected by multiplex PCR, and screen-positive genes were confirmed and sub-classified by PCR. Among 100 cattle investigated, 180 E. coli strains with diarrheic genes (DEC) were detected in 79 cattle, and 45 of them, isolated from 32 cattle, were Shiga toxin-producing E. coli (STEC). More than 30% of cattle carried astA, cdt, cnf and stx2 in descending order. STh gene, LT gene, invE, aggR and afaD were not detected in this study. Both stx1 and stx2 were concurrently detected from 6 of 45 STEC strains and stx2 alone was detected from 19. Seventeen STEC strains carried STp gene, astA, or cdt along with stx1 or stx2. Additionally, 135 remaining DEC were classified into 18 enterotoxigenic E. coli with STp gene, 25 enteropathogenic E. coli with eae, and 92 other DEC with astA, cdt and cnf. Both O and H serotypes were identified in 48 strains, including O157 : H7, O1H7 and so on. O157 : H7 were identified in 3 strains that carried stx2 and eae together, as found in human pathogenic strains isolated from patients with gastroenteritis and hemolytic-uremic syndrome.

Small cell carcinoma of the ovary, hypercalcemic type is a very rare aggressive neoplasm of unknown histogenesis, affecting mainly girls and young women. Recently, inactivating mutations in SMARCA4 (BRG1), a member of the switch/sucrose nonfermenting chromatin remodeling complex, has been identified as driver events in most cases. We herein describe 3 cases in 34, 34, and 37-year-old women. Symptoms were mainly abdominal pain and mass. One patient was normocalcemic, and the other 2 had no preoperative serum calcium values available. All patients received radical hysterectomy with salpingo-oophorectomy, lymphadenectomy, and variable multimodality therapy. Two developed abdominal recurrences/metastases and died of disease at 4 and 12 months. One patient was alive without disease 17 months after surgery and radiochemotherapy. Histologic examination showed undifferentiated neoplasms composed of diffuse sheets, nests and cords of noncohesive monomorphic small blue/basaloid cells (classic variant, 1 case), and large undifferentiated/rhabdoid cells with abundant cytoplasm (large cell/rhabdoid variant, 2 case) admixed with minor small cell areas. One case contained rare isolated goblet cells, but true glandular component was absent. All tumors expressed vimentin and variably pancytokeratin and WT1. Nuclear SMARCB1 was intact in all cases (1 case showed small foci with mosaic loss). All tumors showed complete loss of SMARCA4. In conclusion, SMARCA4 immunohistochemistry represents a highly valuable emerging tool in identifying small cell carcinoma of the ovary, hypercalcemic type in routine practice. Distinguishing this aggressive neoplasm from juvenile granulosa cell tumor and other undifferentiated ovarian cancers is mandatory in selecting appropriate chemotherapeutic regimens and would allow better characterization of this entity, for which targeted molecular therapy still remains to be established.

We report the case of a 67-year-old man with a left pharyngeal tumour, whose peripheral blood showed granulocytosis (white blood cell count, 58 300/μl) and a high serum granulocyte colony stimulating factor titre (184 pg/ml). The tumour showed pleomorphic proliferation of atypical spindle cells in a myxomatous stroma, revealing a sarcomatous pattern. The spindle-shaped neoplastic cells had irregularly shaped nuclei, a thick nuclear membrane, prominent eosinophilic nucleoli and abundant cytoplasm. They strongly expressed wide-spectrum keratin, cytokeratins (CAM5.2, MNF116), vimentin and vascular endothelial growth factor. A few neoplastic cells expressed granulocyte colony stimulating factor. A spindle cell carcinoma was diagnosed. This may be the first documented case of a granulocyte colony stimulating factor producing cancer arising in the pharynx. The patient died four months after the initial symptoms appeared.

A very rare case of hepatoid carcinoma with serous component arising in the fallopian tube of a 79-year-old woman is presented. The lesion was a 5.0-cm unencapsulated, yellowish-white soft mass. The tumor was composed of hepatoid carcinoma (90%) and serous carcinoma (10%) components. The hepatoid carcinoma was histologically characterized by a proliferation of round to polygonal cells arranged in a trabecular, tubular, sinusoidal, papillary, or solid pattern. The serous component in the fallopian tube also showed in situ lesions. Both components showed an infiltration into the surface of the left ovary, omentum, peritoneum including the pouch of the Douglas, and serosa of the colon. Immunohistochemically, the hepatoid carcinoma was positive for alpha-fetoprotein, polyclonal carcinoembryonic antigen (CEA), hepatocyte paraffin 1, albumin, epithelial membrane antigen, and cytokeratin (CAM5.2). Ultrastructurally, the cytoplasm contained abundant ribosomes, moderate amounts of mitochondria, and rough endoplasmic reticulum that developed into a meshwork and contained mitochondria within it. Microbile channel-like structures and desmosomes were occasionally observed. The association with serous carcinoma indicates mullerian origin rather than germ cell origin. The patient received chemotherapy and was alive without disease at 10 months after surgery.

Uterine perivascular epithelioid cell tumor (PEComa) is very rare, and there have been only 27 reported cases. The differentiation of the tumor has been enigmatic, and the biologic behavior remains unclear. This study describes the clinical, histologic, and immunohistochemical features of four cases of uterine PEComa. The patients were 30, 32, 36, and 40 years old, and none of them had tuberous sclerosis complex. The size of the tumors ranged from 1 to 30 cm in diameter, and 3 of the 4 tumors were confined in the uterus. One tumor involved the left ovary and omentum. Microscopically, the tumors were characterized by an epithelioid arrangement of tumor cells, which had abundant clear to eosinophilic pale granular cytoplasm, and one tumor had moderately atypical nuclei. Coagulative necrosis was found in two cases. The mitotic figures ranged from 0 to 11 per 10 high power fields. Immunohistochemically, the tumors were positive for vimentin (4/4), HMB45 (4/4), h-caldesmon (4/4), alpha-smooth muscle actin (3/4), muscle actin (2/4), and desmin (3/4). They were uniformly negative for Melan A, CD10, and S-100 protein. Ultrastructural examinations were performed on one case and revealed no melanosomes, premelanosomes, or evidence of smooth muscle differentiation. One patient died of intestinal metastases 17 months after surgery. The other patients have been well with no evidence of disease 8, 12, and 36 months after surgery. Uterine PEComa should be regarded as tumors with an uncertain malignant potential until long-term outcome data for a larger number of patients become available.

A case of juvenile temporal arteritis, which is a rare vascular lesion in children and young adults, associated with Kimura's disease in a healthy 23-year-old asymptomatic man is described. The patient presented with a painless 2.5 cm nodule with eosinophilia and normal erythrocyte sedimentation rate. Histologically, the left superficial artery showed marked intimal thickening with moderate eosinophilic infiltrates, constriction of the vascular lumen, focal disruptions of the internal elastic lamina and media, moderate eosinophilic infiltrates in the adventia, and absence of giant cells. The subcutaneous tissue surrounding the artery was characterized by lymphofollicular hyperplasia, marked eosinophilic infiltrates in the intra- and extra-follicles with abscess, capillary proliferations, lymphocytic, plasma cell and mast cell infiltrates, and fibrosis in the interfollicular region. Immunohistochemically, reticular, positive IgE staining was observed in the germinal centers. Clinically and histologically, the lesion was consistent with juvenile temporal arteritis associated with Kimura's disease. The findings indicate that both entities are closely related and juvenile temporal arteritis may be secondary to Kimura's disease.

Aims : To evaluate the expression of D2-40 in normal lymphatic endothelium and vascular tumours or tumour-like lesions of the skin and soft tissue. D2-40 is a novel monoclonal antibody to a Mr 40 000 O-linked sialoglycoprotein that reacts with a fixation-resistant epitope in lymphatic endothelium. Methods and results : Formalin-fixed paraffin-embedded sections from 30 normal tissue samples, including skin, soft tissue, stomach, and colon, and 84 vascular tumours or vascular tumour-like lesions were immunostained with monoclonal antibodies to D2-40 and CD31. Normal lymphatic endothelial cells in all normal tissues expressed D2-40. Its positive staining delineated flattened channels or open spaces lined by a single layer of endothelial cells whose lumena were sometimes filled with lymphocytes. Ten of 10 cases of lymphangioma, nine of 10 Kaposi's sarcomas (KSs), one of five spindle cell haemangiomas, one of one reactive angioenodotheliomatosis, one of one vascular transformation of lymph node sinuses, three of three Dabska tumours, one of 10 epithelioid haemangioendotheliomas (HEs) and seven of 15 angiosarcomas were positive for D2-40. Positively staining angiosarcomas were characterized by epithelioid or papillary endothelial cells. Twenty-two non-spindle cell haemangiomas, one retiform HE and one Kaposiform HE, and five glomus tumours were negative for D2-40. In comparison, CD31 was expressed in five of 10 lymphangiomas, nine of 10 KSs, 27 of 27 haemangiomas, three of three Dabska tumours, 10 of 10 epithelioid HEs, 15 of 15 angiosarcomas and one of one each of retiform HE, Kaposiform HE, reactive angioendotheliomatosis, and vascular transformation of node sinuses. Five glomus tumours were negative for CD31. Conclusions : The monoclonal antibody D2-40 is a highly sensitive and specific marker of lymphatic endothelium in normal tissue and a subset of vascular lesions, including KS, Dabska tumour, and lymphangioma. The findings support the concept that these tumours show at least partial lymphatic endothelial differentiation. Subsets of angiosarcomas and HEs show both vascular and lymphatic endothelial differentiation. D2-40 can be used in a panel of markers to classify vascular tumours. There is no requirement for epitope retrieval. This novel monoclonal antibody also has the potential for increasing the accuracy of detection of lymphatic invasion in primary tumours and could be widely applied for this purpose in surgical pathology.

A spindle cell carcinoma arose three years after the seeming excision of a so-called "infiltrative" basal cell carcinoma (IBCC) in the cheek of an 87-year-old Japanese woman. The patent had no history of irradiation. The tumor was composed of short fascicles and whorling arrangements of spindle to polygonal cells without residual IBCC. Immunohistochemically, the tumor was positive for vimentin, cytokeratin 8 & 18, epithelial membrane antigen, and alpha-smooth muscle actin. Ultrastructurally, the tumor cells had tonofilaments and desmosomes. The patient died after a local recurrence with metastatic lesions in the lung and the neck lymph nodes that were indicated by CT scanning and MRI at nine months after diagnosis. This case and others support the concept that spindle cell carcinoma can pursue an aggressive clinical course.

Endometrioid carcinoma of the fallopian tube resembling a female adnexal tumor of probable wolffian origin (FATWO) is a rare, recently delineated, low-grade carcinoma that may be confused with both FATWO and high-grade carcinoma. Only about 20 cases have been reported so far, but it probably represents almost half of the endometrioid carcinomas of the fallopian tube. It has a better prognosis than conventional tubal endometrioid and serous carcinomas. Macroscopically, the tumors form a small, solid, polypoid mass that is usually confined within the tube. Microscopically, the tumors are characterized by proliferations of spindle cells in whorls and closely packed confluent elongated glands with focal tubular, sieve-like, and papillary structures. Unlike FATWO, this variant of endometrioid carcinoma exhibits focal squamous differentiation, intraluminal mucin, mild to moderate nuclear atypia, and occasional mitosis. Unlike FATWO, tumor cells are negative for inhibin-alpha and calretinin.