A rare case of Pleuropulmonary blastoma type III with Immunohistochemical Study.

Here we intend to document a rare case of PPB type III in a 2-year male presenting with an extensive tumor occupying the right hemithorax with immunohistochemical (IHC) study. Pleuropulmonary blastoma (PPB) is a rare variably aggressive, dysodontogenetic, childhood primary intrathoracic malignancy which in up to 25% of cases can be extrapulmonary with attachment to the parietal pleura. It is found in pediatric population under 5 years of age. It was initially proposed as a distinct entity by Manivel et al. in 1988. PPB is a proliferation of primitive mesenchymal cells that initially form air-filled cysts lined by benign-appearing epithelium (type I, cystic). Later on, the mesenchymal cells outgrow the cysts with formation of focal solid areas (type II, solid and cystic) and finally, mainly solid mass (type III, solid PPB).

A rare case of primary giant myxoid liposarcoma of the pericardium.

Primary pericardial neoplasms account for 6.7-12.8% of all primary tumors arising in the cardiac region. Pericardial tumors are most likely to be metastatic and are an extension of the primary tumors from the surrounding structures. Sarcomas of the pericardium are rare. Myxoid liposarcoma (ML) represents about 5% of all the soft-tissue sarcomas in adults. They are usually located in the deep soft tissues of the extremities. There have been less than 20 cases of pericardial liposarcomas reported on PubMed since 1973. Here, we present a rare case of primary giant pericardial myxoid liposarcoma (ML) in a 46-year-old female diagnosed on frozen section and later was confirmed histopathologically.

Rare Case of Primary Clear Cell Carcinoma of the Urinary Bladder.

Cancers of the urinary bladder account for about 3.2% of all cancers worldwide. Clear cell adenocarcinomas (CCAs) of urinary bladder are rare. CCA must be differentiated from benign condition such as nephrogenic adenoma (NA) as well as malignant conditions such as urothelial carcinoma with clear cells, metastasis from ovary and kidney, and urinary bladder myomelanocytic tumor. The diagnosis is based on characteristic histopathological and immunohistochemical features. We present a rare case of CCA of urinary bladder with aggressive clinical behavior in a 72-year-old female.

Stromal expression of CD10 in breast carcinoma and its correlation with clinicopathological parameters.

INTRODUCTION: Breast cancer is the foremost cause of death in women worldwide with more than one million cases occurring annually. AIM: This study was conducted to study the stromal CD10 expression in breast carcinomas (BCa) and its correlation with various prognostic factors such as tumor size, histological grade, lymph node status, estrogen receptor (ER), progesterone receptor (PR), HER2neu, and Ki67 status. MATERIALS AND METHODS: Sixty cases of BCa diagnosed between 2013 and 2015 were included in the study. Stromal expression of CD10 was studied on entire section of selected BCa blocks for all cases. A technique of manual tissue microarray was employed for the analysis of expression of immunohistochemical markers ER, PR, and HER2/Neu and Ki67 in all cases. Results were subjected to statistical analysis. RESULTS: Stromal CD10 positivity was seen in 78.3% cases, out of which 53.3% of cases were strongly positive, and 25.0% cases were weakly positive. Positivity for ER, PR, HER2, and Ki67 was 31.7%, 33.3%, 65%, and 75%, respectively. Stromal expression of CD10 was found to be significantly associated with increasing tumor grade, lymph node status, HER2neu positivity, ER negativity, and Ki67 positivity. CD10 stromal expression was seen mainly in PR negative BCa cases; however, it was statistically insignificant. It was noted that CD10 stromal positivity increased with increasing grade. CONCLUSION: CD10 can be used as an independent prognostic marker and should be included in routine histopathology report. CD10 could act as a potential target for newer drug development.

Immunohistochemical Study of ER, PR, Ki67 and p53 in Endometrial Hyperplasias and Endometrial Carcinomas.

INTRODUCTION: Endometrial carcinoma is the second most common gynecologic malignancy in the developing countries. Endometrial Hyperplasia (EH) is a precursor to Endometrioid Adenocarcinoma (EMAC). A 23% of Atypical Hyperplasias (AEH) progress to EMAC. AIM: This study was undertaken to analyse ER, PR, p53 and Ki67 in EH and endometrial carcinomas and attempt correlation with clinical and histopathological findings. MATERIALS AND METHODS: The present study was conducted over a period of seven years. A manual tissue array technique was employed for cases subjected to IHC. Analysis of the expression of IHC markers (ER, PR, p53, Ki67) in EH and endometrial carcinoma was attempted. Results were subjected to statistical analysis. The results were considered to be significant when the p-value <0.05. RESULTS: A total of 85 cases of EH and 28 cases of endometrial carcinoma were included in the study. EH (75.22%) was more common than endometrial carcinoma (24.78%). Among 28 cases of endometrial carcinomas, EMAC was most common (78.57%) followed by Clear Cell Carcinoma (CCC) (14.28%), and Uterine Serous Carcinoma (USC) (7.14%). ER and PR expression decreased as lesion progressed from EH to EMAC. ER and PR expression was negative in USC and CCC. The p53 expression and mean Ki67 labelling index increased as the severity of lesion increased from EH to endometrial carcinoma. CONCLUSION: The ER, PR, p53, Ki67 IHC markers may be included in every case of endometrial carcinoma to understand the tumour biological behavior which in turn could help individual treatment strategies.

Immunohistochemical Study of MUC1 and MUC5AC Expression in Gall Bladder Lesions.

INTRODUCTION: Immunohistochemical (IHC) markers of mucin family are associated with various Gallbladder Lesions (GBLs). AIM: To study the distribution of GBL with respect to age and sex as well as to analyse the IHC profile of MUC1 and MUC5AC in GBLs and attempt correlation with clinical and histopathological findings. MATERIALS AND METHODS: The present study was conducted over a period of six years. A technique of manual tissue array was employed for cases subjected to IHC using MUC1 and MUC5AC. Results were statistically analysed using software program "The Primer of Biostatistics 5.0". RESULTS: A total of 629 GBL were encountered. Out of 605 of non-neoplastic lesions, 32 (5.29%) expressed MUC1 while 515 (85.12%) cases expressed MUC5AC. Out of 24 cases of neoplastic GBL, 20 cases (83.33%) showed positivity for MUC1 and 9 cases (37.5%) were positive for MUC5AC. The rate of MUC1 expression was significantly higher in Gall Bladder Cancer (GBC) {18GB carcinoma (ca) +3 Carcinoma In Situ (CIS)} (85.71%) than chronic cholecystitis (4.71%). The positive rate of MUC5AC expression was significantly lower in GBC (28.57%) than chronic cholecystitis (87.19%). The percentage of cases showing MUC1 expression increased as the severity of disease progressed from hyperplasia to CIS. The percentage of cases showing MUC5AC expression decreased as the severity of disease progressed from hyperplasia to CIS. CONCLUSION: In this study, 96.18% cases were non neoplastic GBL of which chronic cholecystitis (87.77%) was predominant. 3.81% of the GBL constituted for neoplastic lesions of which 75% were GBC. MUC1 showed higher rates of expression in neoplastic GBL. MUC5AC showed higher rates of expression in non neoplastic GBL. Expression of MUC1 and MUC5AC might be closely related to pathogenesis of neoplastic and non neoplastic GBL.

Clinicopathological Study of Carcinoma of the Ampulla of Vater with Special Reference to MUC1, MUC2 and MUC5AC Expression.

INTRODUCTION: The ampulla of Vater consists of papilla, common channel, distal common bile duct and the distal main pancreatic duct. Ampullary carcinomas arise from two different types of mucosa, reflecting their broad histomorphological spectrum. Adenocarcinomas originating in the ampulla of Vater are classified as having either 'Intestinal' or 'Pancreatobiliary' type of differentiation. Pancreatobiliary type has consistently shown a worse prognosis. Various types of mucins are distributed in normal tissues and gastrointestinal tumours. MUC1, MUC2 and MUC5AC are known to be the most important amongst all. AIM: To study clinicopathological features of different types of carcinoma of ampulla with MUC1, MUC2 and MUC5AC expression. MATERIALS AND METHODS: We included 20 cases of ampullary adenocarcinoma in this study, diagnosed after radical surgery. The histopathological slides were reviewed and the tumours were classified into intestinal type, pancreatobilliary and other types. Representative block of tumours was subjected to immunohistochemical (IHC) study with MUC1, MUC2 and MUC5AC antibodies. Results were subsequently analysed. RESULTS: Pancreaticobiliary type of ampullary carcinoma cases predominated (50%) over intestinal type (25%). Carcinomas of "other types" included one case each of mucinous carcinoma, adenosquamous carcinoma and undifferentiated carcinoma and well differentiated neuroendocrine tumours. Pancreatobiliary type of carcinomas was associated with higher tumour stage and grade and lymph node metastasis. All the pancreatobiliary type expressed MUC1 and all intestinal type carcinomas expressed MUC2. MUC 5AC expression was seen predominantly in pancreatobiliary type of ampullary carcinomas. CONCLUSION: Mucin markers are associated with two different subtypes of ampullary carcinomas which have different prognosis. Immunohistochemical study of mucin expression can define the different types of carcinomas of ampulla of Vater with better objective criteria.

Role of CD10 Immunoexpression in Grading Phyllodes Tumour of the Breast.

INTRODUCTION: Fibroepithelial tumours are a heterogeneous group of biphasic neoplasms consisting of a proliferation of both epithelial and stromal components. Fibroadenoma (FA) and Phyllodes Tumour (PT) constitute the major entities. It is crucial to distinguish benign from borderline PT (low grade malignant PT), because the former do not metastasize, have a lesser risk of local recurrence and initial local recurrences are histologically benign in almost all instances. Multiple Immunohistochemical (IHC) markers are being studied to find their utility in grading the PT accurately for planning proper treatment. AIM: To study, the IHC expression of CD10 in the stromal cells of a series of PTs and FA, with the aim of determining whether the degree of CD10 expression in the stromal cells is related to the grade of the tumour. MATERIALS AND METHODS: Records of 28 cases of PT and 35 cases of FA received in the Department of Pathology in a tertiary care hospital were obtained. Histopathology reports and slides of all the cases were reviewed and clinical data such as age and histomorphological features such as tumour cellularity, stromal overgrowth, mitotic count and nuclear atypia were noted. Representative block of the tumour with maximum cellularity was subjected to CD10 staining. For FA and benign PT a technique of tissue microarray was used. For borderline and malignant PT, representative section was used. Stromal cell staining was assessed, using cytoplasmic staining of the breast myoepithelium as internal control. RESULTS: Present study included 35 cases of FA, 20 cases of benign PT, five cases of borderline PT and three cases of malignant PT. The mean age of the patients increased with the increasing tumour grade of PT and this was also observed for FA and benign PT. The mean age increased with increase in tumour grade of PT and was statistically significant (p<0.05). The mean size did not increase with the increasing tumour grade of PT and was statistically insignificant (p=0.0429). Mean tumour size was more in benign PT as compared to FA and was highly statistically significant (p<0.01). CD10 staining was diffuse (Grade-3) and strong in malignant PT. The staining intensity was strong but patchy (Grade-2) in borderline PT. Weak and patchy (Grade-1) CD10 staining was seen in four benign PT and six FA. Other cases of benign PT and FA were negative for CD10 immunoreactivity. CONCLUSION: Our study showed that CD10 expression strongly correlates with the PT grade, which can help in the differentiation between benign and malignant variants of PT.

Immunohistochemical Study of MUC1, MUC2 and MUC5AC Expression in Primary Breast Carcinoma.

INTRODUCTION: Breast Cancer (BC) is the second most common cancer among women in India and accounts for 7% of global burden of BC and one-fifth of all Cancers (CA) among women in India. AIM: This study was conducted for studying the expression of MUC1, MUC2 and MUC5AC in breast carcinoma. MATERIALS AND METHODS: Fifty cases of primary breast carcinoma diagnosed between years 2013 to 2015 were included in the study. Manual tissue array technique was applied for cases subjected to Immunohistochemistry (IHC). An analysis of the expression of IHC markers (MUC1, MUC2, MUC5AC, ER, PR and HER2/neu) was attempted. Results were subjected to statistical analysis. They were considered to be significant when the p-value was less than 0.05. RESULTS: The positivity for MUC1, MUC2 and MUC5AC in BC was 58%, 8% and 6% and for ER, PR and HER2 was 48%, 36% and 64% respectively. There was a significant correlation between MUC1 expression and ER and PR positivity. There was a significant correlation between MUC2 expression and ER positivity. No significant association was observed between MUC2 and PR expression, MUC5AC expression and ER and PR positivity. There was statistically significant correlation between negative MUC2 and MUC5AC expression and histopathological grade. It was noted that MUC2 and MUC5AC negative tumours were associated with higher tumour stage though not statistically significant. It was noted that MUC5AC negative tumours showed higher frequency of lymphovascular invasion though not statistically significant. CONCLUSION: Our experience with the present study highlights the role of mucins in the development and progression of BC.

A rare case of renal metastasis from squamous cell carcinoma of the cervix.

Cervical cancer is the most frequent type of cancer in women in many developing countries. Squamous cell carcinoma of the cervix spreads principally by lymphatics and less commonly through blood vessels. The most frequent sites for those who develop distant metastasis include lungs (21%), lumbar and thoracic spine (16%), and para-aortic lymph nodes (7%). Metastasis to the kidney is extremely rare with <10 previously reported cases. We report a case of renal metastasis from squamous cell carcinoma of the cervix detected in end-stage kidney due to hydronephrosis.

A rare case of extraskeletal Ewing's sarcoma/primitive neuroectodermal tumor developing in maxillary sinus of an old patient.

Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) family of tumors is an uncommon group of malignant neoplasms that may present in both skeletal and extraskeletal sites. PNET outside the central nervous system is called peripheral PNET (pPNET) developing from migrating embryonal cells of the neural crest. Very few cases of pPNET of the maxilla are reported in English literature. These tumors may be difficult to diagnose due to their primitive morphology. These tumors occur predominantly in infancy or early childhood. The occurrence of extraskeletal ES/PNET in the maxillary sinus in an old age is very rare. We report a case of extraskeletal ES/PNET developing in maxillary sinus in a 60-year-old woman. The ES/PNET should be included in the differential diagnosis of a small round cell tumor and immunohistochemical analysis with a panel of immunomarkers should be done for correct diagnosis and proper treatment.

A rare case of multicentric secretory carcinoma of breast in an adult female with review of literature.

Secretory breast carcinomas (SBCs) are considered one of the rarest types of BCs accounting for <0.15% of all breast cancers. The lesions are typically solitary, but rare multicentric cases have been reported. SBC have characteristic histopathological, immunohistochemical, and electron microscopic findings. Children and adolescents with SBC have a favorable prognosis, but disease seems slightly more aggressive in adults. The characteristic ETV6-NTRK3 molecular alteration, leading to a stable chimeric tyrosine kinase fusion product, may be the target of promising new treatment for this unique BC. We present a rare case of multicentric secretory carcinoma of breast with aggressive clinical behavior in a 75-year-old female.

Unilateral gonadoblastoma with dysgerminoma in normal fertile woman having a child: Extremely rare occurrence with characteristic immunohistomorphology.

Gonadoblastomas (GBYs) are rare gonadal tumors almost always arising from a dysgenetic gonad with a Y chromosome. Very rarely, GBYs appear in otherwise normal women with a history of pregnancy. The typical histological appearance of GBY can be altered by extensive deposition of basement membrane material, calcification, or overgrowth by a malignant tumor. Less than 10 cases have been reported with normal 46XX karyotype. Only six cases of GBY have been described in pregnant women. We present a unique case of GBY with dysgerminoma in a genotypically and phenotypically normal woman with a history of normal pregnancy, absence of virilization, and characteristic immunohistomorphological features.

An Unusual Case of Paraganglioma of the Broad Ligament Presenting as Cystic Mass.

In clinical practice, broad ligament (BL) tumors are seldom encountered. Paraganglioma of the BL is exceedingly rare entity. Here we present an unusual case of broad ligament paraganglioma, presenting as a cystic mass, in a 50-year-old postmenopausal female. A high degree of suspicion along with detailed immunohistopathological work-up is needed for arriving at an accurate diagnosis.

Risk Stratification in Paragangliomas with PASS (Pheochromocytoma of the Adrenal Gland Scaled Score) and Immunohistochemical Markers.

INTRODUCTION: Paragangliomas (PGLs) are rare tumours that arise in sympathetic and parasympathetic paraganglia and are derived from neural crest cells. Presence of metastasis is the only absolute criterion for malignancy. There is no single histo-morphological feature indicating malignant potential and multiple parameters have been proposed to prognosticate the individual case. This includes studies conducted using Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and Immunohistochemical (IHC) markers. AIM: We have studied ten cases of paraganglioma and attempted to correlate the prognosis with multiple clinicopathological variables. MATERIALS AND METHODS: This study was done in a tertiary care general hospital over a period of five years. Available clinical records and histopathology slides of all patients were reviewed. Using Pheochromocytoma of the Adrenal Gland Scaled Score (PASS), we divided the cases into two groups-tumours showing high risk behaviour (PASS≥4) and tumours showing benign behaviour (PASS<4). IHC analysis was done using synaptophysin, chromogranin, S100 and Ki67. We correlated S100 immunoreactivity and Ki67 proliferative index with PASS score. Both PASS score and IHC markers were also correlated with clinical outcome. RESULTS: There were six Pheochromocytomas (PHC) and four Paragangliomas (PGL). Two paragangliomas were retroperitoneal and one each was located in ear (HNPGL) and broad ligament. PASS score was ≥4 in five cases and <4 in five cases. Out of five cases in which PASS was ≥4, three cases showed clinical evidence of malignancy and two cases were benign. All the cases in which PASS was <4 were clinically benign. S100 immunoreactivity was grade 1 in two cases, grade 2 in six cases and grade 3 in two cases. The cases in which S100 immunoreactivity was grade 1 were malignant. One case in which S100 was grade 2 was clinically malignant. Ki67 labeling index was raised (>3%) in two cases, which were malignant correlated with malignant PASS score. CONCLUSION: We conclude that the following clinicopathological parameters should be taken into account for risk assessment of malignant behaviour of paragangliomas- location, size, PASS score, S100 immunoreactivity and Ki67 labeling index.

Study of Immunohistochemical Markers (CK-19, CD-56, Ki-67, p53) in Differentiating Benign and Malignant Solitary Thyroid Nodules with special Reference to Papillary Thyroid Carcinomas.

INTRODUCTION: Solitary Thyroid Nodule (STN) has provoked increased concern owing to higher incidence of malignancy. The inter and intra observer variation in the histomorphological diagnosis of Papillary Thyroid Carcinomas (PTC) may sometimes pose a diagnostic difficulty. AIM: This study was undertaken to analyse immunohistochemical (IHC) markers (CK-19, CD-56, p53, Ki-67) to differentiate between benign and malignant surgically resected STN along with their utility in the identification of PTC. MATERIALS AND METHODS: The present cross sectional study was conducted over a period of 4 years. A technique of manual tissue array was employed for cases subjected to IHC. The primary antibodies used were CK-19, CD-56, p53 and Ki-67. Analysis of the expression of IHC markers (p53, Ki-67) to distinguish between benign and malignant STN was done. Evaluation and correlation of expression of IHC markers (CK-19, CD-56) to determine its utility in reaching definitive diagnosis and assessing prognosis of PTC was tried. Results were subjected to statistical analysis. The results were considered to be significant when the p-value <0.05. RESULTS: Out of the 160 cases of surgically resected STN specimens, 68 cases were non-neoplastic, 24 cases were benign and 68 cases were of malignant tumours (7 cases of follicular carcinoma (FCa), 61 cases of PTC). CK-19 was found to be a sensitive (83.61%) and a highly specific positive marker (100%) for the diagnosis of PTC. The difference in CD-56 expression between PTC and non-PTC group was found to be highly statistically significant. CD-56 was found to be a sensitive (85.86%) and specific (82.25%) negative marker in differentiating PTC from follicular lesions/neoplasms. The difference in p53 expression between the malignant and non-malignant STN cases was found to be highly statistically significant with a sensitivity and specificity 85.29% and 70.65% respectively. The statistical difference in mean Ki-67 Labeling Index (LI) was found to be significant between PTC versus FA, PTC versus non-neoplastic lesions, FA versus FCa and FVPTC versus FA. CONCLUSION: The panel of four IHC markers (CK-19, CD-56, p53, Ki-67) may be used for differentiating doubtful benign STN cases from malignant ones and also for definitive diagnosis of PTC along with histopathological examination.

A rare case of Mayer-Rokitansky-Kuster-Hauser syndrome with multiple leiomyomas in hypoplastic uterus.

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare disorder described as aplasia or hypoplasia of uterus and vagina due to an early arrest in development of mullerian ducts. Women with this syndrome are characterized by the presence of 46 XX karyotype, normal female secondary sex characters, normal ovarian functions, and underdeveloped vagina. The presence of leiomyoma in MRKH syndrome is very rare, and only few cases have been reported in the literature. Here, we report a case of MRKH syndrome with multiple leiomyomas originating from the rudimentary horn of uterus in 25 years married, phenotypically female patient.

Fine-needle aspiration cytology of recurrent epithelioid sarcoma of the foot: Role of immonocytochemistry in definitive diagnosis.

Fine-needle aspirations (FNA) have played a crucial role in the diagnosis and follow-up of malignancies including sarcomas. However, relatively low specificity in the classification of sarcoma is the main limitation of FNA. Epithelioid sarcoma is a rare tumor of soft tissues. Very few individual case reports describing cytological features of epithelioid sarcoma have been documented in the literature. Here, we describe cytological features of epithelioid sarcoma with immunocytohistological correlation.