Small-cell Carcinoma of the Endometrium Presenting as Cushing's Syndrome

Abstract

Abstract. Small-cell carcinoma (SCC) of neuroendocrine type is an uncommon tumor of the endometrium. no previous report has documented Cushing's syndrome due to ectopic aCTH production by SCC of the endometrium. We describe a 56-year-old Japanese woman with SCC of the endometrium and multiple lung metastases presenting as Cushing's syndrome. The patient was referred to our hospital because of general fatigue with facial and leg edema, and multiple nodular lesions in the bilateral lungs on chest X-ray examination. a physical examination revealed that the patient had moon face, buffalo hump, and truncal obesity. Endocrinological examinations confirmed ACTH-dependent Cushing's syndrome. Thoracic computed tomography imaging showed multiple nodular lesions in the bilateral lungs. abdominal magnetic resonance imaging suggested a malignant tumor of the uterus. The patient received a lung tumor biopsy and surgical hysterectomy. The endometrial carcinoma was histologically a SCC admixed with endometrioid adenocarcinoma. The SCC of the endometrium showed immunoreactivity for pro-opiomelanocortin, aCTH, and vimentin, but not for thyroid transcription factor-1. The lung biopsy specimen had the same features. These findings indicated that the SCC originated from the endometrium, and the ectopic aCTH-producing tumor caused Cushing's syndrome. This study provides the evidence that SCC of endometrial origin was an ectopic aCTH-producing tumor causing Cushing's syndrome.

A case of MPO- and PR3-ANCA-negative pauci-immune renal-limited small-vessel vasculitis associated with endometrial neuroendocrine small cell carcinoma

“…On the other hand, the present case occurred in association with endometrial NSCC, which is a rare disease (less than 1 % of all endometrial cancers) [13]. Some cases of endometrial NSCC have been reported to exhibit paraneoplastic syndrome in relation to neurological or endocrinological symptoms because of its neuroendocrinological nature [14][15][16][17]. In terms of renal involvement, one case each of membranous nephropathy [18] and mesangial proliferative glomerulonephritis [19] have been reported, both of which were considered to be immunocomplex deposition disease in relation to tumor-associated antigens.…”

Magnetic Resonance Imaging of Benign and Malignant Uterine Neoplasms

“…[66][67][68][69][70] On pathology, these tumors are characterized by sheets of small densely packed cells, hyperchromatic nuclei, high nuclear-to-cytoplasmic ratio, and positive markers for neuroendocrine differentiation on immunohistochemistry. 66,67,[69][70][71][72] Uterine small cell carcinomas exhibit aggressive behavior, local invasion, extrauterine spread, early lymph node involvement, and distant metastasis at diagnosis. 66,67,[71][72][73] Clinically, the most common symptoms include abnormal vaginal bleeding and pelvic pain.…”

“…66,67,[69][70][71][72] Uterine small cell carcinomas exhibit aggressive behavior, local invasion, extrauterine spread, early lymph node involvement, and distant metastasis at diagnosis. 66,67,[71][72][73] Clinically, the most common symptoms include abnormal vaginal bleeding and pelvic pain. 67,69,73 Uterine small cell carcinomas may produce and secrete metabolically active amines and peptides and cause paraneoplastic syndromes, including syndrome of inappropriate secretion of antidiuretic hormone, retinopathy, Cushing syndrome, and membranous glomerulonephritis.…”

“…67,69,73 Uterine small cell carcinomas may produce and secrete metabolically active amines and peptides and cause paraneoplastic syndromes, including syndrome of inappropriate secretion of antidiuretic hormone, retinopathy, Cushing syndrome, and membranous glomerulonephritis. 66,68,69,71,73 These tumors have been described in a wide age range of women (23-84 years), with a mean age of 60 years. 67,69 The prognosis is poor and most patients present with advanced disease.…”

“…67,69,72,73 MRI features of uterine small cell carcinoma are limited to case reports, and generally speaking indistinguishable from those of other uterine neoplasms. [66][67][68][69][70][71][72][73] Typically, the tumor is seen as an intracavitary mass, frequently associated with diffuse myometrium invasion and extrauterine spread. 66,67,69 On MRI, the tumor usually has low T1 and high T2 signal intensity.…”