A woman in her 60s presented with a 1-year history of progressively numerous seborrheic keratoses and velvety, gray-brown plaques on the face, neck, axillae, and perineum, causing bothersome ptosis and ectropion (Figure). She also reported a sensation of dry mouth and palms, but denied constitutional, gastrointestinal, genitourinary, and respiratory symptoms. Dermatologic examination confirmed the diagnosis of acanthosis nigricans (AN) and was also remarkable for palmar discoloration with accentuated dermatoglyphs, consistent with "tripe palms." The patient had a known medical history of obesity, type 2 diabetes, hypertension and dyslipidemia, but her metabolic disorders alone could not explain such a florid clinical picture. As such, an occult neoplasm was suspected. A thorough laboratory, imaging, and endoscopic workup revealed an enlarged uterus with uterine masses. Further gynecologic examination revealed a 6-cm solid tumor of the cervix consistent with invasive endocervical adenocarcinoma which was confirmed by histopathologic analysis.Acanthosis nigricans is clinically characterized by symmetric, hyperpigmented, velvety or verrucous plaques in intertriginous areas. It is a cutaneous manifestation of internal disease, most often associated with benign conditions (80%), such as obesity, insulin resistance, and diabetes. Less frequently it represents a paraneoplastic dermatosis, called acanthosis nigricans maligna (ANM), which is in most cases associated with abdominal malignant tumors, especially adenocarcinomas, like gastric cancer. 1 This form of AN typically has a rapid onset and extensive skin involvement, and generally affects adults older than 40 years. It can be accompanied by other cutaneous paraneoplastic manifestations, including tripe palms, so called owing to the resemblance of velvety palmar skin to the stomach lining of ruminants, and the "sign of Leser-Trélat", corresponding to the abrupt appearance of multiple seborrheic keratoses. 1,2 Acanthosis nigricans maligna can present concurrently or following tumor detection, but may also precede the diagnosis and serve as an important clinical clue. 1 Its presence should therefore prompt a thorough workup for malignant abnormality, comprising physical examination and comprehensive complementary investigation, including endoscopic and/or imaging methods.Because ANM skin findings usually improve with treatment of the underlying tumor and worsen with disease recurrence or progression, dermatologic examination is crucial for follow-up. 3 This case is a reminder of how the recognition of particular skin findings can lead to the diagnosis of internal malignant tumors. 3 It also represents a rare clinical observation because, to our knowledge, only a few cases of ANM in association with gynecologic tumors-including ovarian, endometrial, and cervical cancershave been reported. [2][3][4]
Summary POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes) is a rare multisystemic disease. Clinical presentation is variable, the only mandatory criteria being polyneuropathy and monoclonal gammapathy in association with one major and one minor criterion. Primary adrenal insufficiency is rarely reported. We describe a case of a 33-year-old patient, in whom the presenting symptoms were mandibular mass, chronic sensory-motor peripheral polyneuropathy and adrenal insufficiency. The laboratory evaluation revealed thrombocytosis, severe hyperkalemia with normal renal function, normal protein electrophoresis and negative serum immunofixation for monoclonal protein. Endocrinologic laboratory work-up confirmed Addison’s disease and revealed subclinical primary hypothyroidism. Thoracic abdominal CT showed hepatosplenomegaly, multiple sclerotic lesions in thoracic vertebra and ribs. The histopathologic examination of the mandibular mass was nondiagnostic. Bone marrow biopsy revealed plasma cell dyscrasia and confirmed POEMS syndrome. Axillary lymphadenopathy biopsy: Castleman’s disease. Gluco-mineralocorticoid substitution and levothyroxine therapy were started with clinical improvement. Autologous hematopoietic cell transplantation (HCT) was planned, cyclophosphamide induction was started. Meanwhile the patient suffered two ischemic strokes which resulted in aphasia and hemiparesis. Cerebral angiography revealed vascular lesions compatible with vasculitis and stenosis of two cerebral arteries. The patient deceased 14 months after the diagnosis. The young age at presentation, multiplicity of manifestations and difficulties in investigation along with the absence of serum monoclonal protein made the diagnosis challenging. We report this case to highlight the need to consider POEMS syndrome in differential diagnosis of peripheral neuropathy in association with endocrine abnormalities even in young patients. Learning points: POEMS syndrome is considered a ‘low tumor burden disease’ and the monoclonal protein in 15% of cases is not found by immunofixation. Neuropathy is the dominant characteristic of POEMS syndrome and it is peripheral, ascending, symmetric and affecting both sensation and motor function. Endocrinopathies are a frequent feature of POEMS syndrome, but the cause is unknown. The most common endocrinopathies are hypogonadism, primary hypothyroidism and abnormalities in glucose metabolism. There is no standard therapy; however, patients with disseminated bone marrow involvement are treated with chemotherapy with or without HCT.
Among premenopausal women with macroPRL the most common complaints were: galactorrhea (23,5%), menstrual irregularities (29,4%), infertility (29,4%), while among those with true hyperPRL they were found in 45%, 50%, and 15% respectively. Some of the patients presented more than one symptom. No symptoms were reported in 22,2% of patients with macroPRL and 14,8% with true hyperPRL. Three male patients had macroPRL and symptoms: erectile dysfunction, infertility or galactorrhea. Two men with true hyperPRL presented erectile dysfunction with gynecomastia and headaches with visual disturbances (Table 2). Macroprolactinemia: prevalence and clinical characteristics in a cohort of hyperprolactinemic patients assessed for macroprolactinemia
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