Subject(s)
Breast Neoplasms, Male/complications , Neurofibromatosis 1/complications , Paget's Disease, Mammary/complications , Aged , Axilla , Biopsy , Breast Neoplasms, Male/pathology , Breast Neoplasms, Male/surgery , Diagnosis, Differential , Humans , Lymph Node Excision , Male , Mastectomy, Radical , Paget's Disease, Mammary/pathology , Paget's Disease, Mammary/surgeryABSTRACT
Multiple endocrine neoplasia type 2B/3 is characterized by multiple mucosal neuromas, a marfanoid appearance, medullary thyroid carcinoma, pheochromocytoma, gastrointestinal ganglioneuromatosis, and thickened corneal nerves. This rare syndrome is inherited in an autosomal dominant pattern. Early recognition followed by appropriate screening and treatment can be life-saving.
Subject(s)
Multiple Endocrine Neoplasia Type 2b/genetics , Adrenal Gland Neoplasms/genetics , Adrenal Gland Neoplasms/pathology , Carcinoma/genetics , Carcinoma/pathology , Cornea/innervation , Ganglioneuroma/genetics , Ganglioneuroma/pathology , Gastrointestinal Neoplasms/genetics , Gastrointestinal Neoplasms/pathology , Humans , Marfan Syndrome/pathology , Multiple Endocrine Neoplasia Type 2b/diagnosis , Multiple Endocrine Neoplasia Type 2b/pathology , Neuroma/genetics , Neuroma/pathology , Pheochromocytoma/genetics , Pheochromocytoma/pathology , Thyroid Neoplasms/genetics , Thyroid Neoplasms/pathologyABSTRACT
Mycosis fungoides and Sézary syndrome, collectively referred to as cutaneous T-cell lymphoma, are non-Hodgkin's lymphomas that initially appear in the skin. Early-stage disease, limited to the skin, is best treated with sequential topical therapies such as topical nitrogen mustard, psoralen phototherapy (PUVA), or total-skin electron beam therapy. Photopheresis is the first line of therapy for the patient with erythroderma. Systemic therapy is generally reserved for patients with refractory disease and patients who initially present with extracutaneous involvement. Although there are several treatment options for cutaneous T-cell lymphoma, there have been few randomized comparative trials.
