Sarcomatoid variant of Xp11 translocation renal cell carcinoma: A rare case report in an adult female.

Xp11 translocation renal cell carcinoma (XPTRCC) is a very rare kidney neoplasm, which has been predominantly reported in young patients. Sarcomatoid transformation in renal cell carcinomas is known. However, its occurrence in XPTRCC is unreported so far in the literature. We report a unique case of sarcomatoid transformation in a XPTRCC in a 23-year-old female, who presented with a huge right-sided renal mass and had metastatic deposits in lungs. Morphologically, clear cell morphology with papillary architecture along with foci of sarcomatoid transformation and rhabdoid differentiation were noted. Immunohistochemistry showed Pax-8 and TFE-3 expression in all components including the sarcomatous areas, whereas CK and EMA were expressed in conventional clear cell component. We present an extremely rare case of sarcomatous transformation in XPTRCC and discuss the case as determined by histopathology and immunocytochemistry. To our knowledge, this is the first case of sarcomatoid transformation XPTRCC being reported in the world literature.

Grossing and reporting of radical prostatectomy specimens: An evidence-based approach.

Radical prostatectomy (RP) constitutes the primary treatment option for patients with clinically localized, biopsy-proven prostate cancer that requires local treatment with curative intent. Accurate reporting of radical prostatectomy specimens is required to guide further risk stratification and management of patients. Hence, for the handling and reporting of RP specimens, a standardized protocol should be followed. Many general pathologists may not be well-versed with the guidelines for the handling of radical prostatectomy specimens. This article discusses a detailed approach to grossing techniques, including specimen description, fixation requirements, gross cut-up, and reporting of the grade and stage of RP specimens. This will enable the pathologist to aid in multidisciplinary management.

Downregulation of MICA/B tumor surface expressions and augmented soluble MICA serum levels correlate with disease stage in breast cancer.

OBJECTIVE: In this study, the profiling of the expression of major histocompatibility complex (MHC) class I-related chain A and B (MICA/B) in human breast cancer tumor tissue, saliva, and urine samples of breast cancer patients and control is carried out. MICA/B is ligand of NKG2D receptor expressed on malignant cells. The release of MICA/B from tumor tissue comprises an immune escape mechanism that impairs antitumor immunity. Based on this literature we explored the potential of soluble MICA (sMICA) as a marker in breast cancer (BC). METHODS: The expression was profiled by using immunohistochemistry (MICA/B), western blot (MICA/B) and ELISA (MICA). RESULTS: The optical density of western blot of MICA/B in different stages of BC illustrated significant difference as per one way analysis of variance and significant difference with stage III and IV by Dunnett's multiple comparisons test respectively. Analysis of sMICA in serum, saliva and urine of BC patients revealed significantly higher levels (median 41.0 ± 4.1 pg/ml in pre-treatment sera, 181.9 ± 1.6 pg/ml in saliva and 90.7 ± 1.7 pg/ml in urine) than in control (median <1.2 pg/ml). The elevated levels of sMICA were related to the cancer stage. CONCLUSIONS: The elevated levels of sMICA were observed in patients with well differentiated cancer while the poor expression of sMICA was observed in patients with poorly differentiated tumors. Tumor immunity is impaired by the release of MICA in the biofluids and may be useful for detection and diagnosis of the stage of BC.

A comparative study of smica in various body fluids of diagnosed cervical cancer patients and healthy women.

OBJECTIVE: Cervical cancer (CC) is a major public health problem in women, and its early detection can help reduce morbidity and mortality. The objective of this study was to compare serum levels of soluble major histocompatibility complex class I-related chain A (sMICA) levels in various body fluids between women diagnosed with CC and healthy women. METHODS: A case-control study was conducted at a tertiary care hospital and a cancer center in Kolhapur, India. Overall, 150 individuals (100 CC patients and 50 healthy women) participated after providing informed written consent. Demographic data, histopathology history, parity, and tumor, node, and metastasis (TNM) staging data were collected. Pap smears, saliva, blood, and urine samples were collected. Pap smears were examined microscopically, and sMICA levels in all samples were determined by enzyme-linked immunoassay (ELISA). RESULTS: The mean age of women with cervical cancer was 49.86±8.18 years. Squamous cell carcinoma (70%) was the most common histological variant in CC patients. Serum soluble sMICA levels differed significantly with parity and TNM staging (P<0.05). Mean levels of sMICA were significantly different in samples (CC cases vs. healthy patients; saliva: 166.721±108.718 vs. 0.039±0.005 pg/mL; urine: 82.921±45.580 vs. 0.010±0.005 pg/mL; serum: 35.756±10.799 vs. 0.039±0.005 pg/mL, P<0.001). CONCLUSION: Levels of sMICA in body fluids can be considered as a diagnostic or prognostic tool to determine disease progression or tumor regression.

Follicular Dendritic cell sarcoma of caecum in a young boy presenting as Intussusception.

BACKGROUND: Follicular dendritic cell sarcoma (FDCS) is a rare tumor derived from follicular dendritic cells (FDC) occurring in lymph nodes and extranodal sites. It is usually regarded as an indolent tumor with a tendency of local recurrence but a low risk of metastasis. Common extranodal sites are liver, lung, tonsil, spleen, soft tissue, and mediastinum. Extranodal FDCS of gastrointestinal tract (GIT) is exceedingly rare, with just 36 cases reported in the literature. METHODS: We report an unusual case of FDCS of caecum in a 13-year-old boy who presented as intussuception. On histology, it posed a diagnostic challenge for us. An inconclusive initial immunohistochemistry (IHC) lead us to suspect FDCS which was confirmed by FDC markers. CONCLUSIONS: The diagnosis of FDCS at extranodal site like GIT is all the more challenging because of its rarity, morphologic heterogeneity, and lack of awareness.

Multilocular cystic renal oncocytoma-Awareness of an unusual morphologic variation.

BACKGROUND: Renal oncocytomas are benign epithelial tumors usually detected incidentally. They are typically solid,well-circumscribed,homogenous,mahoganybrown with a central stellate scar.Sometimes,they can have cystic degenerationand rarely present as a multilocular cyst which can be mistaken for other cystic renal carcinomas. METHODS: We describe a case of incidentally detected multilocular cystic renal oncocytoma having an unusual gross appearance of multiloculation with perinephric fat invasion. The tumor exhibited tubulocystic architecture posed a diagnostic dilemma. Detailed study of multiple sections coupled with immunohisto chemistry helped elucidate the diagnosis. Till date, only eight cases of multicystic renal oncocytoma have been reported in the English literature. CONCLUSIONS: We emphasize the importance of awareness of this unusual morphologic variation to ensure correct diagnosis.

Gross reporting of adult kidney tumor specimens: A comprehensive evidence-based review.

Renal tumors comprise a wide spectrum of benign and malignant tumors. The important prognostic factors in renal cell carcinoma include pathological stage, tumor grade, morphological type, sarcomatoid/rhabdoid differentiation, and tumor necrosis. Therefore, the pathologist needs to be fully aware of how to gross nephrectomy specimens to be able to accurately provide the above prognostic information while reporting adult kidney tumors. With the advent of nephron-sparing surgeries, due diligence should be exercised to assess and sample the parenchymal surgical margin. This article discusses the approach to grossing nephrectomy specimens, elaborates the significance of every step, and also sheds light on the importance of clinical and radiological information in providing a holistic approach to the diagnosis and staging of adult renal tumors.

Alveolar rhabdomyosarcoma of urinary bladder presenting as acute leukemia: A diagnostic trap.

Rhabdomyosarcoma (RMS) masquerading as acute leukemia (AL) is very rare. We report a case which presented as acute leukemia subsequently diagnosed to be Alveolar RMS of Urinary Bladder. Although cases of RMS with leukemic presentation have been reported, to our knowledge this is the first case of Alveolar RMS of Urinary Bladder with leukemic picture at initial presentation. We would like to emphasize that this critical error can have serious consequences on the treatment and outcome of these patients.

Grossing and reporting of testicular tumor specimens : An evidence-based approach.

The majority of testicular tumors are germ cell tumors (GCTs), but there are numerous other types, making testicular tumors one of the most diverse areas of human pathology, despite their relative rarity. Testicular tumors are usually diagnosed only after radical surgery, as biopsies are not performed. Further management of the patient is dependent on the diagnosis at microscopy, which itself is based on the sections taken at the time of grossing the specimen. Many pathologists often aren't well versed with guidelines for handling of orchiectomy specimens and for microscopy. This article discusses, in detail, the approach to grossing of a testicular tumor specimen and elaborates of the reasons as to why we do what we do at the initial "cut-up". It explains the logic behind the reporting guidelines for testicular tumors and offer a clinical primer to the pathologist as to why we do what we do while grossing testicular tumor specimens.

Mammary analogue secretory carcinoma: An Indian experience of a novel entity.

AIMS: To explore clinical, histopathological and immunohistochemistry (IHC) features of mammary analogue secretory carcinoma (MASC) with systematic literature review. SETTINGS AND DESIGN: Hospital based cross-sectional study. SUBJECTS AND METHODS: The data of all cases of MASC diagnosed over a period of 1 year i.e., from July 2017 to July 2018 were retrieved. The haematoxylin and eosin (H and E) sections, and IHC sections were studied. A strict histological and recently updated criteria were applied and patients with a confirmed diagnosis of MASC were included in the study. A systematic literature review was conducted by searching the PubMed and National Centre for Biotechnology Information database. STATISTICAL ANALYSIS USED: Microsoft Excel 2010. RESULTS: The present case series is 27th in the English literature and 1stcase series describing its histopathology in the Indian literature. The mean age of presentation is 43 years. Female preponderance was found i.e., M:F ratio of 0.5. CONCLUSION: Histopathology and if necessary, followed by IHC is required for the confirmation of diagnosis of MASC. We should be aware about this recently described entity which is usually mistaken for other low grade salivary gland carcinomas like Acinic cell carcinoma (AciCC) and Mucoepidermoid carcinoma (MEC). The knowledge about its typical morphology, high degree of suspicion and IHC confirmation with both S-100 and Mammaglobin help in precise diagnosis.

Thyroid cancer in a long-term nonprogressor HIV-1 infection.

Long-term non-progressor HIV infection (LTNP-HIV) is seen in <1 percent of HIV-afflicted population. There are definite criteria for the diagnosis of LTNP-HIV. Malignancies either solid tumors or haematological cancers have not been reported in such population. We report here a rare case of follicular thyroid carcinoma in LTNP-HIV infection. She never had any opportunistic infections. She did not receive anti-retroviral therapy in the entire course of illness and continued to have good quality of life. Treatment of follicular thyroid cancer was similar to other patients without HIV infection. This could be the first case study from India.

AIDS-associated cancers: an emerging challenge.

OBJECTIVES: To study the incidence and effects of anti-retroviral therapy along with cancer chemotherapy on outcome of AIDS associated Cancers in Indian patients. METHOD: 3832 cancers patients were investigated over a period of 5 years. 46 AIDS-associated cancers were identified. HIV status was evaluated by ELISA, Western Blot, viral load and CD4/CD8 counts. Patients were treated with different modalities of cancer management and anti-retroviral therapy was discussed with the patient and relatives. Patients were followed up 6 monthly. RESULTS: Incidence of AIDS-associated cancers was 1.2 percent. AIDS-Defining Cancers (ADC) were seen in 26 (54.35%) while non-AIDS-Defining Cancers (NADC) were observed in 21 (45.65%). Non Hodgkin Lymphoma was the commonest form of AIDS-defining cancers in 21 (84%) patients, cervical cancers in 4 (16%) women while there was not a single case of Kaposi's Sarcoma. AIDS associated cancers were common in males. Mean age was 38.5 years. Only 33.5% patients received treatment for HIV and cancers. Development of immune reconstitution syndrome was observed in 9.09% patients. Hepatitis B infection was seen in only one patient (2.17%). CONCLUSIONS: AIDS-associated cancers are seen in advanced stage of HIV infection. Concurrent chemotherapy and anti-retroviral therapy for ARL is significantly effective. Cervical cancers and non-AIDS-defining cancers do not show predictable response to anti-retroviral therapy. Mortality in non-AIDS related cancers was significantly higher than AIDS related cancers.