D'emblee type of mycosis fungoides of head and neck

Abstract

Mycosis fungoides as a disorder of reticulo-endothelial system occurs in the eczematous or psoriatic stage, progresses to infiltrated plaque stage and finally to tumorous form with or without systemic spread. The head and neck is rarely involved. A case is presented in which the disease process started as a tumor (d'emblee type) and remained localized in the head and neck region without a generalized spread. The tumorous lesion rapidly increased in size and involved deeper tissues of the neck to cause infranuclear facial palsy and medial bulging of the tonsil' it also extended in the superior mediastinum casuing pressure on the trachea and the esophagus. The patient died of respiratory failure.

Solitary CD8-Positive Primary Cutaneous Peripheral T-Cell Lymphoma: A Question of Classification

“…4 Progression of solitary MF to a tumor stage has been described, as an exceptional event, and only in folliculotropic MF. 5,6 Tumor d'emblee is a rare presentation of tumor stage MF, either as solitary or multiple tumors, 7,8 and is of doubtful status because it predates advances in immunohistochemistry, since it has barely, if ever, been seen. Primary cutaneous acral CD8(+) T-cell lymphoma is a new addition to the provisional category in the most recent WHO classification.…”

Surgical Treatment of Obstructive Palpebral Tumors in d'Emblee Variant of Cutaneous T-Cell Lymphoma

“…Based on the clinical course and presentation, our patient's palpebral lesions represent the d'emblee variant of cutaneous T-cell lymphoma. [4][5][6] Our experience suggests that surgical debulking may represent an effective adjunct therapy in selected cases of tumor stage cutaneous T-cell lymphoma that are recalcitrant to other therapies. Anatomic sites known to heal well by second intention (facial concavities and mucosal surfaces) may be particularly amenable to this approach.…”

“…In these rare cases, tumors develop abruptly, without the usual disease progression. 4,5 This presentation has been associated with facial palsy, respiratory failure due to pressure on the trachea, and internal lymphoma. We report a case of sudden-onset, rapidly growing eyelid tumors.…”

“…It was called d'emblee, or sudden type. In these rare cases, tumors develop abruptly, without the usual disease progression 4,5 . This presentation has been associated with facial palsy, respiratory failure due to pressure on the trachea, and internal lymphoma.…”