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Introduction: Dysembryoplastic neuroepithelial tumor (DNT) is a rare benign brain tumor predominantly involving children and young adults. Histologically, it corresponds to WHO Grade I tumors; however, it may masquerade aggressive neural tumors such as oligodendroglioma, oligoastrocytoma, pilocytic astrocytoma, and ganglioglioma. The literature on clinical, radiological, and pathological spectrum of DNT is described mostly in the form of case reports, with only a few case series reported till date.Methods: A retrospective review of files with diagnosis of DNT (2016 to 2018) was made in the Department of Pathology, National Institute of Pathology, New Delhi. A total of ten cases were retrieved, and their clinical, radiological, and histopathological features were reviewed and studied. Special stains and immunohistochemistry were done, wherever required.Results: The mean age was 14.8 (±7.9) years, with a male-to-female ratio of 1.5:1. The most common mode of presentation was recurrent, intractable seizures. The most common site of lesion was parietal lobe followed by temporal and frontal lobes of the brain. On histology, mucoid matrix admixed with floating neurons and oligodendrocyte-like cells was a consistent feature; however, the presence of specific glioneuronal elements was observed in only a few cases.Conclusions: DNT is a benign, low-grade, nonrecurrent neuroepithelial neoplasm. It is important to differentiate this rare entity from other mimickers, as it is surgically curable and carries an excellent prognosis without the need for adjuvant chemotherapy and radiotherapy. The study helps to enrich the clinicopathological aspects of this rare but important entity. [ABSTRACT FROM AUTHOR]

Ganglioneuromas (GNs) are well-differentiated, rare benign tumors of neural crest origin and are, for the most part, considered to be the benign equivalent of neuroblastomas. There are very few cases of GN reported to be at presacral location in the literature. The standard form of treatment is the total surgical excision. However, total resection of GN is not always possible depending on the neuron, from which it originates, and its localization. Moreover, adjuvant radiotherapy (RT) or chemotherapy is not recommended even though patients are still symptomatic after subtotal resection. This view is based on the urban legend that it undergoes a malignant transformation although it is a benign tumor. Moreover, there are no data indicating that the GN cases reported in the literature have undergone RT. Therefore, articles about the suspicion that GN may undergo spontaneous or malignant transformation after RT are absolutely controversial. Based on our case, we present here, we believe that we will explain the valid necessity of application of RT that we administered for the first time and that with the clarification of this controversial topic, a significant gap will be closed in the literature. [ABSTRACT FROM AUTHOR]

Epithelial-myoepithelial carcinoma (EMC) is a biphasic low-grade malignant tumor that shows ductal structures lined by the ductal cells with surrounding myoepithelial cells. EMC is most commonly reported in the parotid gland followed by other major and minor salivary glands. EMC of the tongue is an extremely uncommon tumor, with only five previously published case reports. We herein report the sixth case of EMC of the tongue. A 40-year-old female who had throat pain, dysphagia, and right earache for 3 years showed a 4 cm × 4 cm nodular tumor at the base of her tongue. Histopathological and immunohistochemical features were diagnostic of EMC with cervical lymph node metastasis. The metastatic behavior of EMC of the tongue in the absence of myoepithelial anaplasia or high-grade histological features is unusual and reported in this case report for the first time. [ABSTRACT FROM AUTHOR]

Background and Aim: To investigate the value of peripheral blood plasma levels of septin-9 and clusterin protein in the diagnosis of epithelial ovarian cancer (EOC).Materials and Methods: The peripheral blood plasma samples were obtained from 137 EOC patients, 12 borderline ovarian tumor patients, 10 benign ovarian tumor patients, 41 benign pelvic lesion patients, and 58 healthy women. The peripheral plasma septin-9 and clusterin proteins levels were measured by enzyme-linked immunosorbent assay. The power of test was evaluated with the area under the receiver operating characteristic curve (ROC) (AUC).Results: The mean levels of plasma septin-9 and clusterin in EOC patients were significantly higher than that in healthy women (P = 0.002, P = 0.021). The mean levels of plasma septin-9 in benign pelvic lesion patients were significantly higher than that in healthy women (P = 0.007). The mean levels of plasma septin-9 in epithelial ovarian carcinoma patients with tumor family history or distant metastases were significantly higher than that of patients without (P = 0.040, P = 0.025). The AUC of septin-9 protein was 0.712, when the optimal cut-off point was 0.28, the sensitivity and diagnostic specificity were 82.5% and 50.0%, respectively; the AUC of clusterin was 0.636, and when the optimal cut-off point was 87.96 ng/ml, the sensitivity and diagnostic specificity was 71.5% and 41.4%, respectively.Conclusion: The plasma levels of septin-9 and clusterin in ovarian cancer patients were abnormally elevated, which might be used as potential candidates of peripheral blood tumor biomarkers for early diagnosis of EOC and septin-9 might be related to distal metastases of EOC. The septin-9 might play the promotion role, which protein level relates to not only the distal metastases but also the prognosis of EOC. Due to the limit of sample volume, further enlargement of the sample size and set up of the follow-up system is in need to in-depth study the relationship between plasma protein concentration with the distal metastases, and further explore its correlation with the prognosis of EOC. [ABSTRACT FROM AUTHOR]

Introduction: The odontogenic keratocyst is known for its aggressiveness, high recurrence rate, and transformation of keratinized epithelia to nonkeratinized squamous epithelium for which inflammation has been suggested to be responsible. B-cell lymphoma/leukemia-2 (Bcl-2) an anti-apoptotic protein, prolongs the life span of epithelial cells and allows proliferation, differentiation, and morphogenesis.Materials and Methods: Study was carried out comprising of 90 cases; (30 ameloblastoma, 30 keratocystic odontogenic tumor (KCOT), and 30 radicular cyst). Bcl-2 expression was determined with respect to localization, area (percentage) and intensity of stained cells in epithelium, and connective tissue stroma by counting the endothelial, round, and fusiform cells.Results: In epithelium bcl-2 expression in KCOTs was higher followed by ameloblastoma and lowest in the radicular cyst. Whereas, in connective tissue stroma bcl-2 expression was higher in KCOT and radicular cyst than ameloblastoma cases. Solid variants showed statistically higher expression as compared to the unicystic variants of ameloblastoma (P = 0.009, 0.033, 0.011, and 0.041).Conclusion: High expression of bcl-2 in KCOT supports the general agreement that some features of KCOT are those of a neoplasia. The bcl-2 expression in connective tissue cells suggests that these cells may also be important as epithelial cells in the biological behavior odontogenic keratocyst. [ABSTRACT FROM AUTHOR]

Introduction: Ductal carcinoma in situ (DCIS) is considered a heterogeneous lesion at the molecular level. However, there is a paucity of literature about the existence of molecular subtypes in DCIS which can predict their biological behavior at the preinvasive stage.Materials and Methods: Precise prevalence of molecular subtypes of pure DCIS and DCIS component of infiltrating duct carcinoma (IDC) was evaluated using immunohistochemistry and correlated with known prognostic factors.Results: DCIS cases were classified as luminal A (46.6% in each group), luminal B (pure DCIS 20% and DCIS component of IDC 13.3%), HER2 overexpressing, basal and nonbasal (pure DCIS 3.3% and 26.6% and DCIS component of IDC 3.3% and 33.3%, respectively), and triple negative, nonbasal (pure DCIS and DCIS component of IDC 3.3% each). The molecular phenotype of DCIS correlated well with that of the coexisting IDC.Conclusions: This study demonstrated molecular heterogeneity in DCIS; however, similar molecular phenotypes were seen in the coexisting IDC suggesting that DCIS is a precursor lesion and can predict phenotype of the invasive component. This also suggests that the invasiveness of DCIS is not dependent solely on the molecular character of the tumor epithelial cells, but factors such as tumor microenvironment may play a role. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study was to investigate the value of two quantitative indicators, the apparent diffusion coefficient (ADC) and the exponent apparent diffusion coefficient (EADC), of magnetic resonance imaging (MRI) diffusion-weighted imaging (DWI) in the differential diagnosis of ovarian epithelial tumors. Materials and Methods: Clinical and MRI data from ovarian epithelial tumors were analyzed after pathology confirmation of 85 lesions from 76 cases (47 lesions from 41 benign cases; 38 lesions from 35 malignant cases). Patients underwent routine MRI examination and DWI before surgery. The average ADC and EADC values of the solid sections of the tumors were measured when the b value was 1000 s/mm 2. Results: The mean ADC value of the solid sections in the benign group was 1.28 ± 0.23 × 10-3 mm 2/s; the average EADC value was 27.96 ± 5.78 × 10-2. In the malignant group, the mean ADC value of the solid sections was 0.86 ± 0.17 × 10-3 mm 2/s; the average EADC value was 42.37 ± 5.96 × 10-2. When the b value was 1000 s/mm 2, there was a statistically significant difference in ADC and EADC values between benign and malignant ovarian tumors (P < 0.05). Conclusion: ADC and EADC values of DWI can be used to differentiate benign and malignant ovarian epithelial tumors. [ABSTRACT FROM AUTHOR]

Background: Fine needle aspiration cytology (FNAC) is a useful diagnostic modality in the evaluation of solitary thyroid nodules (STN). It can differentiate between benign and malignant lesions in most cases.Aim: This study was undertaken to determine the utility and diagnostic accuracy of FNAC in the evaluation of STN.Materials and Methods: In this retrospective study, a total number of 483 thyroid FNACs were retrieved, out of which 209 cases of STN were chosen for this study. The Bethesda system for reporting thyroid cytopathology (TBSRTC) was used for analysis. Their FNACs diagnoses were compared with histopathological diagnoses.Results: Among 209 FNACs, 88 (42.1%) had non-neoplastic lesions, 6 (2.9%) had atypia of undetermined significance/follicular lesions of undetermined significance (AUS/FLUS), 52 (24.9%) had follicular neoplasm/suspicious for a follicular neoplasm (FN/SFN), 33 (15.8%) were suspicious for malignancy and 18 (8.6%) had malignant cytology. The sensitivity, specificity, accuracy, positive predictive value and negative predictive value of FNAC in STN cases were 94.4%, 97.6%, 95.8%, 98.1% and 93.2% respectively.Conclusion: Our study concluded that FNAC reporting using TBSRTC highly correlated with the histopathological diagnosis and our results were comparable with published data. The FNAC diagnosis helps in triaging patients with STN and identifies those who require surgical intervention. It is a simple, convenient, cost effective, sensitive, specific, safe and accurate initial diagnostic method for the preoperative evaluation of STN. [ABSTRACT FROM AUTHOR]

Perivascular epithelioid cell tumor (PEComa) is a group of sarcomas that exhibit a myomelanocytic phenotype and possess a unique cell type in the perivascular epithelioid cell. Traditionally HMB-45 immunoreactivity is the first criteria required to consider a tumor to be PEComa. We report a case of multifocal PEComa with negative HMB-45 marker. The patient presented with three big ulceroproliferative lesions; two over right thigh and one over the scalp in the right frontal region. The patient was prescribed with oral sirolimus to which good response was seen. To the best of our knowledge, this is the first case of HMB-45 negative multifocal malignant PEComa from India. [ABSTRACT FROM AUTHOR]

Adenosquamous carcinoma is a rare malignant epithelial neoplasm characterized by the presence of both areas of squamous cell carcinoma and adenocarcinoma. Only few cases of oral adenosquamous carcinoma have been previously reported in the literature. It has been described as a squamous cell carcinoma subtype with a high infiltrative capacity. This paper reports a rare case of adenosquamous carcinoma that involved the upper left buccal mucosa in a 55-year-old man. [ABSTRACT FROM AUTHOR]

Surface epithelial tumors of the ovary comprise over fourth of the ovarian neoplasms. Serous adenofibromas are lesser known variants of serous surface epithelial tumors. Though these tumors have a benign fate, yet they can be misinterpreted clinically and radiologically due to their borderline or malignant gross morphological as well as clinical presentation. The cell of origin of surface epithelial tumors of the ovary is histological debated; however, it is now ascertained to have origins from the ovarian cortical and surface lining. Adenofibromas are known to progress in an indolent manner with metachronous behavior for years. The present case is of an ovarian serous adenofibroma in a young female masquerading as peritoneal carcinomatosis. [ABSTRACT FROM AUTHOR]

Desmoplastic small round cell tumor (DSRCT) is rare and highly malignant neoplasm. DSRCT affects usually young males but can occur in adults also. Intra-abdominal pelvic region is the preferred site. Though confirmed by histology, immunohistochemistry (IHC) plays a key role in diagnosis. IHC profile is characteristic, it shows simultaneous expression of epithelial (epithelial membrane antigen (EMA) and cytokeratin (CK)), muscular (desmin), and neural (neuron-specific enolase) markers. Many cases of DSRCT are diagnosed as poorly differentiated carcinoma due to lack of proper panel of IHC. It is difficult to predict if there has been a true increase in incidence. Prognosis is uncertain in such an aggressive neoplasm as chemotherapy (CT) or radiotherapy (RT) shows various outcomes. Here in; we report four cases, all of which showed diagnostic dilemma and uncertain prognosis. [ABSTRACT FROM AUTHOR]

Aim: To report the outcome of carcinoma of the uterine cervix patients treated radically by external beam radiotherapy (EBRT) and high-dose-rate (HDR) intracavitary radiotherapy (ICRT).Materials and Methods: Between January 1997 to December 2001, a total of 550 newly diagnosed cases of carcinoma of the uterine cervix were reported in the department. All cases were staged according to the International Federation of Gynecologists and Oncologists (FIGO) staging system, but for analytical convenience, the staging was limited to stages I, II, III, and IV. Out of the 550 cases, 214 completed radical radiotherapy (EBRT + HDR-ICRT) and were retrospectively analyzed for presence of local residual disease, local recurrence, distant metastases, radiation reactions, and disease-free survival.Results: There were 7 (3.27%), 88 (41.1%), 101 (47.1%), and 18 (8.4%) patients in stage I, II, III, and IV, respectively. The median follow-up time for all patients was 43 months (range: 3-93 months) and for patients who were disease free till the last follow-up it was 59 months (range: 24-93 months). The overall treatment time (OTT) ranged from 52 to 73 days (median 61 days). The 5-year disease-free mean survival rate was 58%, 44%, 33%, and 15%, with 95% confidence interval of 48 to 68, 37 to 51, 24 to 35, and 6 to 24 for stages I, II, III, and IV, respectively. There were 62 (28.97%) cases with local residual disease, 35 (16.3%) developed local recurrence/distant metastases, 17 (7.9%) developed distant metastases, and 9 (4.2%) had local recurrence as well.Discussion and Conclusion: The overall outcome was poor in advanced stage disease, but might be improved by increasing the total dose, decreasing overall duration of treatment, and by adding chemotherapy in patients with disease limited to the pelvis. [ABSTRACT FROM AUTHOR]

Lung cancer combined with eczema is a rare disease. We report a case of 58-year-old man with eczema as the first manifestation of a lung cancer. Skin examination revealed diffuse erythema, dander, itchy rash, and scratch. Chest contrast-enhanced computed tomography showed a heterogeneously enhanced irregular mass in the right lung. Punch biopsy of the tumor confirms squamous cell lung cancer. Eczema vanished nearly completely after one cycle of chemotherapy. [ABSTRACT FROM AUTHOR]

Myoepithelial tumors are most commonly seen as salivary gland tumors. Tumors of similar morphology and nomenclature are also seen rarely in soft tissue, skin, lungs and breast. Bone is an uncommon anatomical site for occurrence of myoepithelial tumors. Histologically, they have variable admixture of epithelial elements in a gamut of patterns with myxoid matricial background. Most of these are benign with very anecdotal reports of malignant counterpart, myoepithelial carcinoma. Herein we describe an extremely rare case of a malignant myoepithelial tumor arising from the rib which owing to unusual location and immunohistochemical profile was diagnostically challenging. [ABSTRACT FROM AUTHOR]

Primary mucinous epithelial tumors of the testis are extremely rare. Although isolated case reports and small series have been published, these interesting neoplasms are less well-known. We report a case of a primary intratesticular mucinous cystadenoma in an asymptomatic 44-year-old man. Right radical orchiectomy was performed because a malignant testicular tumor was suspected. We discuss the management of this uncommon testicular tumor based on the limited reports. [ABSTRACT FROM AUTHOR]

Malignant kidney tumors are rare neoplasms accounting for 3% of adult malignancies. Majority of these arises in the renal parenchyma and are adenocarcinomas. Malignant mesenchymal tumors of kidney are extremely rare. We report on the clinical behavior, the radiological and histopathological details of one such case of malignant mesenchymal tumor, we encountered. [ABSTRACT FROM AUTHOR]

Thymomas are epithelial neoplasm of thymus and most common primary neoplasm of anterior and superior mediastinum affecting males and females equally. It occurs usually in the fifth to seventh decade. Approximately one-third of thymomas are invasive. Metastases to distant extrathoracic sites such as the liver, lung, lymph node, kidneys, ovary and brain occur infrequently. This is more common with invasive thymomas. Although brain has been shown to be a site of infrequent metastases, intraorbital metastases has not yet been reported. Here we report one such case of invasive thymoma of anterior superior mediastinum, which later metastasized to orbit as well as the brain. [ABSTRACT FROM AUTHOR]