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.

Malignant recurrent orbital solitary fibrous tumor.

Solitary fibrous tumor (SFT) is a rare mesenchymal tumor of fibroblastic origin commonly occurring in pleura. It can occur at many extrapleural sites but is rare in orbit. Most cases are benign and recurrence is not unusual in the head and neck and orbit and is usually due to incomplete surgical excision. However, malignant transformation (MT) in orbital SFT is extremely unusual. We present a case of orbital SFT in adult male who developed recurrence with MT eight years after initial surgical excision. He underwent left orbital exenteration. The recurrent tumor revealed features of malignancy with areas exhibiting morphology typical of SFT. The immunochemistry confirmed the diagnosis of SFT with MT. The patient was given adjuvant radiation and was disease free for the last 18 months. Identification of malignancy in orbital SFT is important for the patient to receive appropriate postoperative treatment, as seen in the present case.

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.

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.

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.

Tumor budding - A promising prognostic histopathological parameter in oral squamous cell carcinoma - A comparative immunohistochemical study.

INTRODUCTION: The majority of the head and neck squamous cell carcinomas (HNSCC) occur in the oral cavity. Even with advances in cancer therapy only minor improvements in the survival of HNSCC patients have taken place and approximately 350,000 patients die annually of HNSCC worldwide. Tumor budding (TB) is a novel and promising histo-morphological parameter that has been studied in many cancers. The presence of TB is associated with lymph node and distant metastasis as well as poor survival, independently of the applied scoring system. The depth of tumor invasion (D) measured from the surface of the tumor to the deepest point of invasion is also an important prognostic parameter for oral squamous cell carcinoma (OSCC) with a cutoff point of 4 mm. Both taken together constitute BD model and it has also been found to be an independent prognostic factor for patients with OSCC. Therefore, it would be highly beneficial to evaluate TB and BD model in routine histopathological reporting. AIMS AND OBJECTIVES: This study aims to compare the detection of TB in hematoxylin-eosin and pan-cytokeratin stained immune-histochemical sections of OSCC and also to evaluate whether BD score can serve as a reliable prognostic indicator for OSCC. METHODOLOGY: A total of 30 formalin-fixed, paraffin-embedded tissue blocks of clinically and histopathologically diagnosed cases of OSCC were retrieved. One section was stained with hematoxylin and eosin and the other was processed for pancytokeratin immunohistochemistry to evaluate tumor buds. Depth of invasion (D) was also evaluated to achieve the BD score. RESULTS: Statistical significance (P < 0.001) was noted between TB score evaluated in hematoxylin and eosin (H&E) and pancytokeratin stained sections. There was no statistical significance between age, gender, site of lesion, clinical staging, survival and BD score. CONCLUSION: Immunohistochemical analysis of TB is superior to H&E staining in detection of tumor buds at the tumor invasive front.

Epithelioid sarcoma of paratesticular region: A case report of an unusual case.

Primary tumors of the paratesticular region are rare, with sarcomas constituting a major proportion, particularly in the old age. The paratesticular region consists of mesothelial, various epithelial, and mesenchymal cells and may therefore give rise to a variety of tumors. Epithelioid sarcoma usually occurs at the distal extremities of young adults. The proximal-type variant is characterized by a more aggressive course and resistance to treatment. We herein report a case of 32 years male with scrotal swelling diagnosed as proximal-type epithelioid sarcoma of paratesticular region after histopathological examination and immunohistochemistry. To the best of our knowledge, this is only the second case of proximal epithelioid sarcoma of paratesticular region in the English literature. As these tumors are rare, it has been difficult to establish their optimal treatment. Also, these can be easily misdiagnosed as other epithelioid lesions.

Ultrasonography - A diagnostic modality for oral and maxillofacial diseases.

BACKGROUND: Many diseases present themselves in oral and maxillofacial regions and various modalities may be applied for their diagnosis, including intraoral and panoramic radiography, ultrasonography (USG), computer tomography, magnetic resonance imaging, and nuclear medicine methods such as positron emission tomography. Of these modalities, USG is easy to-use for the detection of non-invasive and soft tissue related diseases in oral and maxillofacial regions. USG plays an important role in analyzing normal and abnormal structures. In particular, in oral and maxillofacial regions, the USG may be clinically applied to evaluate lymph nodes, subcutaneous, and oral cavity-related diseases. AIMS: The aim was to correlate the findings of USG and histopathology for the diagnosis of oral and maxillofacial pathology and to evaluate whether USG can be used as an adjunct in diagnosing oral and maxillofacial pathology. MATERIALS AND METHODS: A total of 10 clinically diagnosed patients with intraoral cancerous growths, swellings in maxillary and neck region were included in this study. Incision biopsy was obtained for confirming provisional clinical diagnosis. The selected cases were advised USG. All patients were then posted either for hemi-glossectomy, hemi-mandibulectomy, and partial maxillectomy with or without radical neck dissection. STATISTICAL ANALYSIS: Student's t-test and coefficient of correlation was used to statistically analyze significant relationship of both the methods. RESULT: In all 10 cases, USG correlated well with histopathology findings, it could also delineate tumor extent and measure tumor thickness. CONCLUSION: USG is an excellent method for the diagnosis of soft tissue lesions and can be used as an adjunct in diagnosing oral and maxillofacial pathology.

Quality assessment program in histopathology: a pilot study from Maharashtra.

Histopathology reports are important quality assurance tools and evaluation of pathological diagnoses described in them is an integral part of total quality control and quality improvement program. We describe a program based on slide circulation which was aimed at both continuing education to upgrade knowledge and proficiency testing of histopathologists. The performance of the participating pathologists was analyzed and the degree of agreement was also studied. The results showed improvement indicated by rising level of performance in 35.3% of consistent participants and increasing trend in the average score. The degree of agreement was comparatively low (65.29%). The practicability of this program and its acceptability as an EQAS was also investigated.