Primary Gastric Synovial Sarcoma in a Child: A Case Report and Review of the Literature.

Synovial sarcoma is a mesenchymal neoplasm that shows a specific t(X;18) translocation that leads to the formation of SS18-SSX gene fusions and is most commonly seen in soft tissues of the extremity. The gastrointestinal tract is a very rare site of involvement. We report a case of primary gastric synovial sarcoma in a 13-year-old male child. Synovial sarcoma should be included in the differential diagnosis when spindle cell neoplasms are encountered in the stomach. A high degree of suspicion, followed by the necessary immunohistochemistry and molecular studies, is required to make an accurate diagnosis.

Operative Outcomes of Minimally Invasive Esophagectomy versus Open Esophagectomy for Resectable Esophageal Cancer.

Background There is a recent rise in the incidence of esophageal carcinoma in India. Surgical resection with or without neoadjuvant chemoradiation is the current treatment modality of choice. Postoperative complications, especially pulmonary complications, affect many patients who undergo open esophagectomy for esophageal cancer. Minimally invasive esophagectomy (MIE) could reduce the pulmonary complications and reduce the postoperative stay. Methodology We performed a retrospective analysis of prospectively collected data of 114 patients with esophageal cancer in the department of surgical oncology at a tertiary cancer center in South India between January 2019 and March 2020. We included patients with resectable cancer of middle or lower third of the esophagus, and gastroesophageal junction tumors (Siewert I). MIE was performed in 27 patients and 78 patients underwent open esophagectomy (OE). The primary outcome measured was postoperative complications of Clavien-Dindo grade II or higher within 30 days. Other outcomes measured include overall mortality within 30 days, intraoperative complications, operative duration and the length of hospital stay. Results A postoperative complication rate of 18.5% was noted in the MIE group, compared with 41% in the OE group ( p = 0.034). Pulmonary complications were noted in 7.4% in the MIE group compared to 25.6% in the OE group ( p = 0.044). Postoperative mortality rates, intraoperative complications, and other nonpulmonary postoperative complications were almost similar with MIE as with open esophagectomy. Although the median operative time was more in the MIE group (260 minutes vs. 180 minutes; p < 0.0001), the median length of hospital stay was shorter in patients undergoing MIE (9 days vs. 12 days; p = 0.0001). Conclusions We found that MIE resulted in lower incidence of postoperative complications, especially pulmonary complications. Although, MIE was associated with prolonged operative duration, it resulted in shorter hospital stay.

Tall Cell Carcinoma with Reverse Polarity of Breast: Report of a Case with Unique Morphologic and Molecular Features.

Breast carcinomas are a heterogeneous group of malignancy, having variable clinical outcomes depending on their cellular and molecular properties. Tall cell carcinoma with reverse polarity (TCCRP) is a recently described rare entity, which shares morphological features with tall cell variant of papillary thyroid carcinoma but has a distinct morphological, immunohistochemical, and molecular profile. We describe a case of a 40-year-old female patient, who presented with lump in the breast. The patient underwent lumpectomy and was diagnosed as tall cell carcinoma with reverse polarity. Immunohistochemistry and bi-directional Sanger sequencing for IDH2 mutation were used for diagnosis. Tall cell carcinoma with reverse polarity is a rare and newly described entity with characteristic morphological and molecular findings, which carries an excellent prognosis.

Spectrum of metastatic neoplasms of the brain: A clinicopathological study in a tertiary care cancer centre.

BACKGROUND: While brain metastases (BM) are the most common causes of neurologic disorders in patients with known systemic malignancies, they can often be the initial manifestations of an undetected primary elsewhere. BM are major causes of morbidity and mortality in cancer patients. AIMS: We describe a mixed population (data from both retrospective and prospective collection) having a BM from a solid tumor. We report the percentage distribution of the most frequent types of BM, confirming the data published in the literature. This paper may play a role in presenting the Southeast Asian reality compared with the Western countries. SETTING: A tertiary-care cancer centre. MATERIALS AND METHODS: Data for 4 years were retrieved from the records of the Department of Pathology of our institute. Hematolymphoid and meningeal tumors were excluded. Hematoxylin and eosin (H and E) stained slides were reviewed, and in cases with an unknown primary, immunohistochemistry (IHC) was advised. The panel of markers was chosen based on the histomorphology on H and E sections. IHC was done in cases with an unknown primary where paraffin blocks were available. RESULTS: Lung cancer was found to be the most common primary malignancy (n = 30; 48.4%) followed by breast cancer (n = 13; 21%), colorectal cancer (n = 6; 9.6%), and skin cancer (melanoma) [n = 3; 4.8%]. CONCLUSION: The incidence of BM from lung and breast cancer was similar to that seen in the Western studies. However, BM from colorectal cancer and melanoma show a higher and lower incidence, respectively, in comparison with the Western literature.

Pediatric Hodgkin lymphoma in a South Indian regional cancer center: its immunomorphology, tumor-associated macrophages, and association with Epstein-Barr virus.

Pediatric Hodgkin lymphoma (HL) comprises approximately a fifth of all patients with HL in India. Seventy-four cases of pediatric classical Hodgkin Lymphoma (cHL) from a regional cancer center in southern India were analyzed on a tissue microarray (TMA) for the stage of B-cell differentiation of the Hodgkin/Reed Sternberg (HRS) cell by immunohistochemistry (IHC) using CD10, bcl6, MUM1/IRF4, and CD 138. Fifty-two of seventy-four (70.3%) cases were of late germinal center/early post-germinal center phenotype (CD10-/bcl6-/MUM1+/CD138-). Epstein-Barr virus (EBV) association using Epstein-Barr virus encoded RNA (EBER) RISH and EBV-LMP1 immunohistochemistry (IHC) revealed an EBV association of 93%. Tumor-associated macrophages (TAM) in the microenvironment were also assessed on the TMA by CD68 IHC, and most cases (59.7%) showed >25% TAMs, with no case showing ≤5%. These findings indicate that pediatric cHL in India is a tumor, predominantly, of late germinal center/early post-germinal center B cells, is almost invariably EBV associated, and with a high number of TAMs in the microenvironment. This latter finding suggests that criteria other than TAM scores need to be developed for risk stratification of pediatric EBV-associated HL especially in developing countries.

Epigenetic alteration of p16 and retinoic acid receptor beta genes in the development of epithelial ovarian carcinoma.

Silencing of tumor suppressor and tumor-related genes by promoter hypermethylation is one of the major events in ovarian carcinogenesis. In this study, we analyzed aberrant promoter methylation of p16 and RAR-ß genes in 134 epithelial ovarian carcinomas (EOCs), 23 low malignant potential (LMP) tumors, 26 benign cystadenomas, and 15 normal ovarian tissues. Methylation was investigated by methylation-specific PCR (MSP), and the results were confirmed by bisulfite DNA sequencing. Relative gene expression of p16 and RAR-ß was done using quantitative reverse transcriptase PCR (qRT-PCR) on 51 EOC cases, 9 LMP tumors, and 7 benign cystadenomas with 5 normal ovarian tissues. Aberrant methylation for p16 and RAR-ß was present in 43 % (58/134) and 31 % (41/134) in carcinoma cases, 22 % (05/23) and 52 % (12/23) in LMP tumors, and 42 % (11/26) and 69 % (18/26) in benign cystadenomas. No methylation was observed in any of the normal ovarian tissues. The mRNA expression level of p16 and RAR-ß was significantly downregulated in EOC and LMP tumors than the corresponding normal tissues whereas the expression level was normal in benign cystadenomas for p16 and slightly reduced for RAR-ß. A significant correlation of p16 promoter methylation was observed with reduced gene expression in EOC. For RAR-ß, no significant correlation was observed between promoter methylation and gene expression. Our results suggest that epigenetic alterations of p16 and RAR-ß have an important role in ovarian carcinogenesis and that mechanism along with methylation plays a significant role in downregulation of RAR-ß gene in ovarian cancer.

Primary pulmonary rhabdomyosarcoma in children: Report of three cases with review of literature.

Primary pulmonary rhabdomyosarcoma in children is rare. Three children aged three, nine and three years were evaluated for abnormal shadows on radiological examination with pneumothorax in two cases. Resection and histopathological examination revealed embryonal rhabdomyosarcoma in all and cystic malformation in first case. All the three children were treated with surgery and first two received adjuvant chemotherapy. The disease free duration was 160 months, 19 months and seven months respectively. The literature on primary pulmonary rhabdomyosarcoma in children was reviewed.

Fetal rhabdomyoma of the lower extremity.

We report a rare case of cellular fetal rhabdomyoma in a 9-year-old male, in the unusual location of right lower thigh. These tumors are more common in the head and neck region; and this case the second such case to be reported in the thigh. Fetal rhabdomyoma is a benign tumor of the skeletal muscle, showing varying degrees of skeletal muscle maturation. The present report discusses the histopathological features, the differential diagnosis and the importance of making the correct diagnosis for proper management of this rare entity.

Myxoinflammatory fibroblastic sarcoma--report of a rare case at an unusual site with review of the literature.

We report a case of myxoinflammatory fibroblastic sarcoma in a thirteen year old girl who presented with a tender swelling in the left upper back. The tumor consisted of varying proportions of inflammatory, myxoid and hyalinized areas. Large bizarre cells with virocyte like inclusions and lipoblast like cells were present. To the best of our knowledge this is the first reported case of myxoinflammatory fibroblastic sarcoma of the back, the extremities being the commonest site of involvement. Due to its varied histologic appearance, the tumor should be differentiated from various benign and malignant soft tissue lesions.

Ovarian sex cord tumour with annular tubules diagnosed by fine needle aspiration cytology--a case report.

Sex cord tumor with annular tubules (SCTAT) is a rare ovarian neoplasm with distinctive morphological features whose cytological features have rarely been described. We report a case of SCTAT diagnosed on fine needle aspiration cytology (FNAC) with a brief review of literature. A 35 year old female presented with recurrent abdominal mass. Fine needle aspiration was done under ultra sound guidance. Cytology smears showed three dimensional tubular structures, metachomatic hyaline masses and uniform neoplastic cells dispersed and in cohesive clusters. Cell block study showed the characteristic simple and complex tubules leading to the diagnosis. The cytological findings of a rare ovarian neoplasm are described, which is only the third such report in English literature. The cytologic features of ovarian SCTAT are as distinctive as its histologicfeatures. Cell block study brings out the distinctive architecture of the tumor and helps in differentiating it from other closely related tumors.

Primitive neuroectodermal tumor of the kidney. A report of two cases diagnosed by fine needle aspiration cytology.

BACKGROUND: Primitive neurocetodermal tumors (PNETs) constitute a family of neoplasms of presumed neuroectrodermal origin most often presenting as bone or soft tissue masses. There are very few reported cases of PNET of the kidney and none diagnosed by fine needle aspiration cytology (FNAC), to the best of our knowledge, in the world literature. We present two cases of renal PNET diagnosed on cytology. CASES: Two patients with renal masses were diagnosed as having PNET on FNAC. Cytologically the tumors showed a dispersed population of malignant small round cells with focal rosette formation and perivascular arrangement of tumor cells. Immunohistochemistry on the cell blocks in both cases showed strong membrane positivity for CD99 (MIC2). Cytogenetic studies in both cases showed the characteristic t(11;22)(q24;q12) translocation, with additional chromosomal abnormalities in case 2. CONCLUSION: PNET of the kidney is a distinct entity and can be diagnosed on fine needle aspiration smears and confirmed with immunohistochemistry and cytogenetic studies. A diagnosis of PNET must be included in the differential diagnosis of renal masses in adolescents and young adults.