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Carcinoid Syndrome Caused by an Atypical Carcinoid of the Uterine Cervix

Developmental Endocrinology Branch, National Institute of Child Health and Human Development (C.A.K., G.P.C.), National Institutes of Health, Bethesda, Maryland 20892; Departments of Endocrinology and Metabolism (T.E.K., C.H.T., S.C.C.), Pathology (N.A., M.A.F.), and Radiology (R.C.J.), Georgetown University Medical Center, Washington, DC 20020; and Department of Endocrinology and Metabolism (T.M.O.), The Ohio State University Medical Center, Columbus, Ohio 43210

Address correspondence and requests for reprints to: Christian A. Koch, M.D., Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 10N262, 10 Center Drive, MSC 1862, Bethesda, Maryland 20892-1862.

Neuroendocrine tumors of the cervix are rare and are often under- or misdiagnosed. Because these tumors are very aggressive, early diagnosis and subsequent treatment are warranted. We describe a 46-yr-old woman with carcinoid syndrome caused by an atypical carcinoid of the uterine cervix. At age 44, she had dysplasia on Pap smear and underwent total abdominal hysterectomy with the diagnosis of adenocarcinoma. Fourteen months postoperatively, she developed the carcinoid syndrome and was found to have numerous liver metastases. Histological and immunohistochemical investigations of biopsy specimens from the patient’s liver lesions and original cervical lesion ("adenocarcinoma") suggested that this woman had a primary atypical carcinoid of the uterine cervix with metastases to the liver. Treatment with octreotide and alkylating agents decreased the episodes of flushing and diarrhea within 8 weeks. If an adenocarcinoma of the uterine cervix is diagnosed, atypical carcinoid should be in the differential diagnosis. Symptoms of the carcinoid syndrome should be pursued and, if present, a urinary 5-hydroxyindolacetic acid level should be obtained. Timely diagnosis of a neuroendocrine tumor of the cervix may improve survival.

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