A large mediastinal benign myoepithelioma effacing the entire hemithorax: case report with literature review

BACKGROUND: Myoepithelial neoplasms, although sometimes encountered in soft tissues are described very rarely in mediastinum and lung. We reported a rare case of such a tumor which was very large in size and not connected to respiratory tree. CASE PRESENTATION: A 24 year old male presented with blunt chest pain and respiratory distress. A CT scan was performed which showed large heterogeneously enhancing soft tissue mass occupying the left hemithorax. It measures 18.5 X 15.8 X 7.6. Thoracotomy with excision of the tumor was done. Operative findings include multilobulated and nodular large glistening white tumor located in anterior mediastinum adherent to parietal pleura and effacing the pulmonary parenchyma. However tumor was not connected or seems to originate from trachea or lung. Microscopic sections show neoplastic lesion composed of nests, cords and trabeculae of small to medium sized cells with round nuclei and clear cytoplasm. Background showed myxoid appearance with areas of cartilaginous differentiation. Immunohistochemical expression of CKAE1/AE3, p63, ASMA, S100 and GFAP favored the diagnosis of benign myoepithelioma. CONCLUSION: Myoepithelial tumors are rare soft tissue tumors thought to arise from stem cells capable of divergent differentiation and occur anywhere in the body. Histopathologic recognition of these tumors is essential as these tumors may behave in a benign fashion despite large sizes

Frequency of translocation t(11;22)(q24;q12) using fluorescence in situ hybridization (FISH) in histologically and immunohistochemically diagnosed cases of ewing\u27s sarcoma

Introduction: Ewing sarcoma (ES) family of tumors is one of the most common groups of malignancies arising in children, adolescents, and young adults. Although characteristic histology with immunohistochemical expression of CD99 and FLI1 after exclusion of other small round blue cell tumors is considered diagnostic of ES, frequency of typical ES translocation, i.e., t(11;22)(q24;q12) is not known in our population. Therefore, in this study, we aimed to evaluate the frequency of this translocation in histologically and immunohistochemically diagnosed cases of ES along with its association with other pathological parameters.Methods: A total of 43 morphologically and immunohistochemically diagnosed cases of ES were included in the study. Fluorescence in situ hybridization (FISH) was performed on representative paraffin blocks to identify t(11;22)(q24;q12) translocation. Association with various clinicopathological characteristics was determined.Results: Mean age of the patients was 18.23±9.57 years. Bone was the most commonly involved site (22; 51.2%) followed by soft tissue (17; 39.5%) and parenchymal organs (4; 9.3%). A total of 88.4% of cases were found to be FISH-positive for t(11;22)(q24;q12). No significant association of translocation positive cases was noted with tumor size or disease-free survival. Similarly, no significant association of tumor size with disease-free survival was found.Conclusions: A significant proportion of cases of histologically diagnosed cases of ES exhibited characteristic t(11;22)(q24;q12). This signifies that histology along with immunohistochemistry is reliable for the diagnosis of this tumor; however, in difficult cases, FISH can be performed to detect characteristic translocation. Moreover, we did not find tumor size to be a significant prognostic indicator of survival in ES

Clinicopathologic features and prognostic grouping of gastrointestinal stromal tumors (GISTs) in Pakistani patients: An institutional perspective

Objectives: Gastrointestinal stromal tumors (GISTs) are rare tumors of gastrointestinal tract, prognosis of which largely depends upon histopathologic characteristics of resection specimens, which were not widely studied in our population. Therefore we aimed to evaluate the histopathologic characteristics of GISTs in our population and their prognostic grouping according to college of American pathologist’s guidelines. Results: Mean age of patients was 53.4 years (18–71 years). 92% of cases were of primary GISTs and stomach was the most common site (57.7%). 75% of cases were of spindle cell morphology and 53.8% belonged to high risk prognostic group. Comparison of stomach and intestinal GISTs showed that intestinal GISTs were found to be of high grade (70%) and of high risk prognostic group (75 and 80%) compared to stomach GISTs (43% were of high risk prognostic group), however this fnding was not statistically signifcant. GISTs are infrequent gastrointestinal tumors but early diagnosis and identifcation of adverse histological features are key to successful treatment. We found a large majority of GISTs to be located in stomach, however intestinal GISTs were found more likely to be associated with adverse prognostic parameters. However more large scale studies are warranted to establish this fnding

Clinicopathological features of craniopharyngioma: A 15-year study from a tertiary care center in Pakistan

Introduction: Craniopharyngiomas (CPs) are benign neoplasms and most common suprasellar tumors. They are more frequent in children, contributing to a significant number of intracranial tumors in the pediatric population and are thought to be arising either from the epithelial remnant cells of the craniopharyngeal duct or from the adenohypophysis epithelium. Two subtypes of CPs exist, namely, adamantinomatous craniopharyngioma (ACP) and papillary craniopharyngioma (PCP). ACP is more common in children with a relatively aggressive clinical course and more frequent relapses than PCP. The study objective was to evaluate the clinicopathological features of CP in our population.Methods: We conducted a retrospective observational study in the Department of Histopathology at Aga Khan Hospital, Karachi, Pakistan, over a period of 15 years, from January 2001 to December 2015. All CP cases were included in the study. A total of 207 cases were diagnosed during this period by histopathologists based on histologic features. All slides were retrieved, and diagnosis was confirmed after a reexamination of slides.Results: We found that the mean age of diagnosis was 25.59±14.71 years, and the median follow-up time was 7 (3-19) years. The number of male patients was 136 (65.7%) and the number of female patients was 71 (34.3%). The most common tumor site was suprasellar (71.5%) followed by the sellar and temporal lobe (12.1% and 6.8%, respectively). The most common complaints were headache (21.7%), followed by loss of vision/decreased vision (16.4%) and vomiting (5.3%). The overall survival rate was 95.2% with a recurrence rate of 5.8%. A significant association of survival was noted with tumor recurrence.Conclusion: CP is a rare brain tumor with good overall survival. We found a low recurrence rate of CP in our study. However, recurrence was found to be the most important factor determining survival in patients with CP

Role of peritoneal washing cytology in ovarian malignancies: correlation with histopathological parameters

BACKGROUND: Peritoneal dissemination of ovarian tumors is a major prognostic parameter in ovarian malignancies. Analysis of peritoneal washing cytology serves as a useful predictor of ovarian surface involvement and peritoneal metastasis even in the absence of clinical omental spread. The aim of the current study is to correlate peritoneal cytology with various histologic features of ovarian cancers in our setup. METHODS: A total of 60 cases of ovarian tumors were included in the study that underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and omental and lymph node sampling during 2009 till 2014 at the Liaquat National Hospital, Karachi. Any free abdominal fluid was aspirated at the time of surgery. In the absence of free fluid, peritoneal washing was done with 50–100 ml of normal saline. Four cytospin preparations were done along with a cell block preparation. Correlation of peritoneal cytology with various histologic parameters was performed. RESULTS: Out of the 60 cases of ovarian tumors involved in the study, 56 were surface epithelial tumors, 2 germ cell tumors, and 2 metastatic carcinomas. The mean tumor size was 9.6 cm. Capsular invasion was seen in 61 % of the cases, and omental metastasis in 51 % of the cases. Serous carcinoma was found to have a significantly higher frequency of positive peritoneal cytology (76.9 %) compared to endometrioid and mucinous carcinomas (44 and 25 %, respectively). A significant positive correlation was seen between positive peritoneal cytology and capsular invasion and omental metastasis with a p value of <0.001. CONCLUSIONS: Positive peritoneal washing cytology has been implemented in ovarian cancer guidelines because of its prognostic significance in ovarian tumors. In addition to being an indicator of peritoneal metastasis, positive cytology also correlates with capsular invasion and histologic type in ovarian tumors. Therefore, it should always be used as an adjunctive tool in the surgical management of ovarian tumors

Morphological spectrum of vesiculobullous skin lesions: An institutional perspective

Introduction: A vesiculobullous lesion of the skin encompasses a group of dermatological disorders with protean clinicopathological features. They usually occur as a part of the spectrum of various infectious, inflammatory, drug-induced, genetic, and autoimmune disorders. Therefore, accurate diagnosis of these lesions is essential for appropriate management and to reduce the associated morbidity and mortality. The conventional skin punch biopsy is the mainstay in the diagnosis of dermatological diseases, especially when combined with confirmatory tests, such as direct immunofluorescence (DIF). Our study evaluated the clinicopathological spectrum of vesiculobullous lesions.Methods: We studied 150 cases of vesiculobullous lesions at the Department of Histopathology, Liaquat National Hospital and Medical College Karachi, Pakistan. Written and informed consent was taken from the patients followed by skin punch procedure in which three biopsies were obtained, which included one biopsy from the lesion and two peri-lesional biopsies. One peri-lesional biopsy was sent in cryomatrix for DIF studies, whereas the other two were sent in formalin to follow the standard tissue-processing protocol.Results: Our results showed that most patients belonged to the geriatric age group of more than 50 years (44.7%), and 54.7% of the patients were females. Total 74.7% of the patients had generalized lesions, followed by lower limbs (9.3%) and trunk (7.3%) involvement. Most patients were diagnosed with bullous pemphigoid (31.3%), followed by pemphigus vulgaris (27.3%), dermatitis herpetiformis (15.3%), Darier’s disease (14.7%), pemphigus foliaceus (4.7%), epidermolysis bullosa (2%), linear immunoglobulin A dermatosis (2%), paraneoplastic pemphigus (0.7%), and drug reactions (0.7%). DIF studies were applied on 60 cases, out of which complement protein C3c was the most commonly deposited protein (53.3%).Conclusion: Our study emphasized the diagnostic role of skin punch biopsy in the proper evaluation of vesiculobullous skin lesions. Histopathology is the cornerstone diagnostic tool in this regard, with DIF being a useful adjunct

Metaplastic breast carcinoma: Clinicopathological parameters and prognostic profile

Introduction: Metaplastic breast carcinoma (MBC) is defined as breast cancer with a heterologous non-glandular component. MBC is considered a special type of breast cancer with a prognosis that is worse than invasive ductal carcinoma (IDC) of the breast. MBC is the most common breast cancer with a triple-negative profile. Therefore, in this study, we evaluated the clinicopathological parameters, recurrence and survival of MBC in our population.Methods: We conducted a retrospective observational study in the Department of Histopathology at Prince Faisal Oncology Centre, Buraidah, Saudi Arabia, over a period of five years. All cases diagnosed as MBC were included in the study. Estrogen receptor (ER), progesterone receptor (PR), and human epidermal growth factor receptor 2 (HER2/neu) immunohistochemistry (IHC) was performed on representative tissue blocks.Results: Total 183 cases of MBCs were included in the study, out of which 120 cases were excision specimens. The mean age of the patients was 48.84±12.99 years, and the most common age group was between 36 and 50 years of age. Most of the cases were tumor (T) stage T3 (50%), and nodal metastasis was present in 40% of cases. Most cases were grade III (78.7%). ER, PR and HER2/neu positivity was noted in 15.8%, 13.1%, and 9.8% cases, respectively. Follow-up data were available for 70 cases, with a median follow-up period of 4 (1-7) years. Tumor recurrence was noted in 31.4% cases, with a survival rate of 71.4%. Squamous, chondroid, spindle cell differentiation, and matrix production were noted in 70.5%, 7.1%, 13.7%, and 2.2% cases, respectively. A significant association of squamous differentiation was noted with HER2/neu positivity. An inverse association of spindle cell differentiation was seen with axillary metastasis. Survival analysis by Kaplan-Meier revealed a significant association of survival with tumor recurrence.Conclusion: MBC is an important subtype of breast cancer, histopathological identification of which is challenging, owing to varied histological differentiation. We found squamous differentiation to be the most common in MBC, which was associated with HER2/neu positivity. A high recurrence rate of MBC was also observed in our study that was significantly associated with survival

Clinicopathologic features of invasive metaplastic and micropapillary breast carcinoma: comparison with invasive ductal carcinoma of breast

Objective: The aim of this study was to determine the frequency of metaplastic breast carcinoma and invasive micropapillary carcinoma in our population and also to compare the clinico-pathologic features of metaplastic breast carcinoma and invasive micropapillary carcinoma with invasive ductal carcinoma, not otherwise specified (IDC, NOS).Result: 86.9% of the cases were identified as ductal carcinoma, NOS, while 2.2% were metaplastic and 0.76% cases were micropapillary carcinoma. Metaplastic carcinomas were found to be of higher grade as compared to IDC, NOS as 81% of metaplastic carcinoma were grade III compared to 35% IDC, NOS. 79% of metaplastic carcinoma were ER negative and 86% were PR negative, respectively as compared to ductal carcinoma NOS, which were 40% ER negative and 54% were PR. Similarly, 86.7% micropapillary cancers were ER positive and 73.3% were PR positive. Moreover, 66.7% micropapillary carcinoma showed nodal metastasis and 77.8% showed lymphovascular invasion, which was significantly higher than that of IDC, NOS micropapillary and metaplastic carcinomas accounts for less than 2 and 1% of the breast cancer burden in our population and highly correlates with poor prognosis parameters therefore, require more intensive management in our population