Primary hepatic pleomorphic liposarcoma: Case report and literature review / Liposarcoma pleomorfo hepático primario: presentación de un caso y revisión bibliográfica

Primary hepatic liposarcoma is an extremely rare malignant tumour derived from adipocytes and is part of the group of mesenchymal tumours. We present the case of a 43-year-old Hispanic male patient with a pleomorphic hepatic liposarcoma and absence of MDM2 gene amplification. Two years and six months after surgery, the patient is asymptomatic. The present case is the first report of this entity with positive immunohistochemical testing for p16, p53, S100, vimentin and absence of MDM2 gene amplification. (AU)

El liposarcoma hepático primario es un tumor maligno extremadamente raro, derivado de adipocitos, y forma parte del grupo de tumores mesenquimales. Presentamos el caso de un paciente masculino de 43 años con diagnóstico de liposarcoma hepático pleomorfo con ausencia de amplificación del gen MDM2. Dos años y 6 meses después de la cirugía el paciente se encuentra asintomático. El presente caso es el primer informe de esta entidad con estudio inmunohistoquímico positivo para p16, p53, S100, vimentina y ausencia de amplificación del gen MDM2. (AU)

Primary hepatic pleomorphic liposarcoma: Case report and literature review.

Primary hepatic liposarcoma is an extremely rare malignant tumour derived from adipocytes and is part of the group of mesenchymal tumours. We present the case of a 43-year-old Hispanic male patient with a pleomorphic hepatic liposarcoma and absence of MDM2 gene amplification. Two years and six months after surgery, the patient is asymptomatic. The present case is the first report of this entity with positive immunohistochemical testing for p16, p53, S100, vimentin and absence of MDM2 gene amplification.

Melanocytic nevus of the anal canal and granular cell tumor of the cecum: a case report and literature review of 2 coincidentally co-occurring neurocristopathies.

Granular cell tumors are predominantly benign soft tissue tumors originating from Schwann cells, whereas melanocytic nevi are benign proliferations of melanocytes. We present the case of a patient with the presence of both entities located in the cecum and anal canal, respectively, constituting an extremely rare coincidental finding. A 43-year-old woman was evaluated by colonoscopy for iron-deficiency microcytic anemia that had lasted for 1 year. Colonoscopy demonstrated a macular lesion of 0.3 cm with a melanocytic appearance in the anal canal; at the cecum level, a subepithelial, yellowish, and partially mobile firm nodular lesion measuring 1.3 cm was observed. A histopathological study showed a melanocytic nevus in the anal canal and a granular cell tumor in the cecum. This is the first reported case of a patient with the extremely rare coincidental-incidental finding of these 2 entities at the same time.

Primary splenic histiocytic sarcoma associated with hemophagocytic lymphohistiocytosis: A case report and review of literature of next-generation sequencing involving FLT3, NOTCH2, and KMT2A mutations.

BACKGROUND: Histiocytic sarcoma is a very rare monocyte/macrophage-derived hematopoietic system tumor with a poor prognosis whose diagnosis is pathologically challenging due to its extreme rarity and histological overlap with various mimicking entities in which histiocytes also predominate. CASE: We report the case of a 33-year-old male patient with hemophagocytic lymphohistiocytosis, purpuric syndrome, and significant splenomegaly. The patient underwent splenectomy; subsequent macroscopic examination revealed a spleen weighing 2065 grams with hyperemic red pulp and multiple infarcts at the periphery. The histological and immunohistochemical study established a diagnosis of primary splenic histiocytic sarcoma with frequent hemophagocytosis. Next-generation sequencing demonstrated mutations in FLT3, NOTCH2, and KMT2A, microsatellite stability, and a tumor mutational burden of 2 mut/Mb. The patient's condition deteriorated clinically from the appearance of the first symptoms and he died 6 months later from multi-organ failure. CONCLUSION: Primary splenic histiocytic sarcoma is one of the rarest tumors of the hematopoietic system. We report the first case with mutations in FLT3, NOTCH2, and KMT2A, and associated hemophagocytic lymphohistiocytosis.

Primary cutaneous diffuse large B-Cell lymphoma, leg type mimicking subcutaneous panniculitis-like T-cell lymphoma in a COVID-19 setting: case report and review of literature.

Primary cutaneous diffuse large B-cell lymphoma, leg type is a rare entity accounting for 4% of all primary cutaneous lymphomas whose clinical presentation encompasses a range of possibilities. COVID-19 has caused a delay in diagnosis of malignant neoplasms and consequently, this has resulted in poorer prognoses. A 62-year-old woman presented with two smooth-surfaced, mobile, well-circumscribed, oval, skin-colored nodules approximately one-cm in diameter with nonerythematous borders on the lower third of the left leg. Two months later, eleven nodules measuring between one and 1.5cm with erythematous halo, slight scaling, central erosion, and crusting had appeared. Histological study showed moderate pericapillary lymphocytic infiltration in the papillary and reticular dermis and prominent diffuse proliferation of medium to large cells in the subcutis. These exhibited irregular vesicular nuclei, a conspicuous solitary nucleolus of two to three small nucleoli, and three mitoses per high power field. Adipocytes were consistently encircled by neoplastic lymphocytes. Primary cutaneous diffuse large B-cell lymphoma, leg type is a high-grade lymphoma that can manifest as a diagnostic challenge and requires adequate immunohistochemistry and in situ hybridization studies for proper diagnosis, treatment, and prognosis.