Mycosis Fungoides dengan Tampilan Tumor D’Emblee: Laporan Kasus Langka

Abstract

Introduction: Cutaneous T-cell lymphoma (CTCL) is a rare malignancy with mycosis fungoides (MF) being the most common subtype. Classic MF typically presents with patches that may progress over years into plaques and tumors. In rare instances, the tumor d'emblee variant can be found, with initial appearance of tumors without prior patches or plaques. Case: A 37-year-old male presented with a one-month history of multiple cutaneous nodules involving the face, scalp and trunk, accompanied by a five-month history of progressive left testicular enlargement. There was a history of weight loss and severe anemia. Physical examination revealed multiple firm, grouped tumors with smooth surfaces, brownish to violaceous in color, distributed across the scalp, face, back, neck, thorax, abdomen, and bilateral upper extremities. Laboratory findings included pancytopenia, electrolyte imbalance, hypoalbuminemia, and elevated urea and creatinine. Histopathological examination showed diffuse infiltrates of large atypical lymphoid cells with atypical mitoses from superficial to deep dermis. The patient deteriorated rapidly during hospitalization and died three days after diagnosis was established. Conclusion: Tumor d'emblee is a rare and aggressive MF variant with a lower survival rate than the classic MF. It is crucial to establish a diagnosis based on clinical appearance, histopathological examination and immunohistochemistry.