ABSTRACT Metastases in the oral cavity are rare and comprise approximately 1% of all oral malignancies. They usually involve the jaws but may also be found in the soft tissues and salivary glands. Women's most common metastatic malignancies are from primary breast cancers. However, metastasis of mucinous breast carcinoma to the lower alveolus mimicking an aggressive primary malignancy as the initial presentation is exceptionally uncommon. We describe the case of a 66-year-old lady with an ulceroproliferative growth in the right lower alveolus. The lesion eroded the mandible and involved the adjacent soft tissues with no prior history of lesion anywhere else. The lesion clinically mimicked a squamous cell carcinoma and masqueraded as a salivary gland mucinous adenocarcinoma on histopathology. The possibility of a metastatic lesion from the breast rather than a primary of the alveolus was also entertained, aided by the immunohistochemical findings of positivity of the tumor cells for GATA3. A positron emission tomography (PET) scan was undertaken to ascertain the primary site. It detected a hypermetabolic lesion in the left breast, which biopsy revealed mucinous breast carcinoma on histopathological evaluation. Metastasis of breast mucinous carcinoma by the hematogenous route is extremely rare; very few cases have been reported. This case illustrates the diagnostic challenges such a lesion can pose to the surgeon and the pathologist. In the advent of such lesions being the initial clinical presentation, a vigilant clinicopathological and radiological assessment is essential to detect the primary.

ABSTRACT B-cell prolymphocytic leukemia (B-PLL) is an extremely rare disease, accounting for approximately 1% of the lymphocytic leukemias. B-PLL generally occurs in older people. It is characterized by the presence of more than 55% prolymphocytes in the peripheral blood (PB), no or minimal lymphadenopathy, massive splenomegaly, and very high white blood cell counts. The prognosis of B-PLL patients is generally poor, with a median survival of 3 years, although a subset of patients may show a prolonged survival. Herein, we report a case of a 70-year-old male with weakness, generalized lymphadenopathy, and moderate splenomegaly at the initial presentation. Hematologic examination revealed lymphocytic leukocytosis, favoring a chronic lymphoproliferative disorder (CLPD). The key to decoding the precise CLPD was a combination of the clinical profile, morphologic findings on the peripheral blood and the bone marrow, immunophenotypic analysis, and cytogenetic study. The best diagnosis proffered was a de novo chronic lymphocytic leukemia/prolymphocytic leukemia. There was no prior history of lymphoproliferative disorder or lymphocytic leukocytosis. Discriminating this entity from other lymphoproliferative disorders is crucial as the treatment and prognosis are varied compared to the other lymphoproliferative disorders. The diagnostic conundrum encountered and the incredible utility of ancillary studies in such a scenario are highlighted in this study.

ABSTRACT Angiosarcoma is a rare neoplasm, constituting only 2% of all the soft tissue tumors and most frequently involves the skin of the head and neck region in elderly males. They are extremely aggressive tumors with high rates of metastasis and poor outcomes. We report a unique case of angiosarcoma involving an unusual site – upper alveolus and maxilla in a young patient highlighting the diagnostic challenges in such a scenario. A 29 years old female presented with a non-healing wound of the oral cavity, which had progressed to the current maximum size of 6.4 cm within one month. Magnetic resonance imaging (MRI) scan revealed the involvement of maxilla up to the floor of the orbit and adjacent soft tissue. However, no distant metastasis was detected on Positron Emission Tomography (PET) scan. Biopsy of the lesion showed an irregular, highly pleomorphic, and mitotically active epithelioid soft tissue tumor conclusively diagnosed as angiosarcoma.

ABSTRACT Oncocytoma of the eyelid is a rare neoplasm. Oncocytoma associated with an ocular surface squamous neoplasm, namely conjunctival intraepithelial neoplasia, is very hard to find in the literature. Herein we report a case of a 53-year-old male who presented with a swelling in the right lower lid over the last 6 years, along with a growth in the conjunctiva of the same eye for the last 2 years and encroaching upon the cornea for the last 4 months. Excision biopsy of the lower lid mass showed histopathological features consistent with oncocytoma. The conjunctival tissue revealed conjunctival intraepithelial neoplasia 3 (severe dysplasia). This case documents a rare synchronous dual ocular neoplasia, a very unlikely coexistence of oncocytoma with conjunctival intraepithelial neoplasia.

ABSTRACT Langerhans cells, found in the supra-basal region of the mucous membranes in the epidermis of the skin, in lymph nodes and thymus, function as antigen-presenting cells within the histiocyte system. Tumors derived from Langerhans cells (LC) can be divided according to the degree of cytological atypia and clinical behavior into Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS). LCS is rare, and the nodal presentation is even rarer with challenging histological characteristics. LCS has a dismal overcome despite intensive chemotherapy. Herein, we report a case of a 29-year-old male who presented with generalized lymphadenopathy initially considered as a lymphoma. An outright definitive diagnosis could not be attained in the initial histomorphological and immunohistochemical evaluation, fraught with differential diagnoses. The key to decoding the precise neoplasm was a combination of the cytopathologic features, review of the histomorphology, and extensive immunohistochemical assessment in conjunction with the clinical and positron emission tomography (PET) scan findings. The best diagnosis proffered was a Langerhans cell histiocytosis progressing to Langerhans cell sarcoma. This case highlights the grey zone areas in LC neoplasms, the diagnostic conundrums encountered, the indispensable role of meticulous pathological analysis, and the importance of ancillary studies in hammering out the final diagnosis.