Extrapulmonary Small Cell Carcinoma Treatment & Management

Updated: Apr 16, 2015
  • Author: Irfan Maghfoor, MD; Chief Editor: Jules E Harris, MD, FACP, FRCPC  more...
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Medical Care

No randomized studies exist to guide decisions regarding management of extrapulmonary small cell carcinoma. The organ and site of involvement, as well as extent of disease, are important in management. Based on experience published in the form of retrospective reviews, combination chemotherapy appears to form the mainstay of treatment, similar to that for small cell carcinoma of the lung. [7] A study by Grossman et al found that surgery and radiation significantly improve median, 5-year, and 10-year survival rates, although outcomes remain poor. [8]

Patients with extrapulmonary small cell carcinoma who present with localized disease may be treated with chemotherapy and local therapy in the form of surgery or radiation therapy. Those presenting with extensive-stage disease are best treated with combination chemotherapy. The active regimens include those containing platinum (cisplatin or carboplatin) or anthracyclines. Combination chemotherapy with a platinum-based combination has produced response rates similar to those seen in small cell lung cancer, and long-term survival has been reported. [9]

Patients with extrapulmonary small cell carcinoma and extensive stage disease should be treated initially with combination chemotherapy. The role of surgery and radiation therapy in this situation is not defined, but surgery may be used for palliative purposes. Those who achieve complete remission may have prolonged survival despite presenting with advanced-stage disease. Survival in excess of 120 months has been reported.

The optimum therapy for limited-stage extrapulmonary small cell carcinoma is less clearly defined, but the principles of management of limited-stage small cell carcinoma have been frequently applied in the management of limited-stage extrapulmonary small cell carcinoma. Surgery, radiation, and chemotherapy may play a role in the management. In contrast to small cell lung cancer, surgery is often the primary therapy in such individuals since the presentation in organs such as esophagus, thyroid, and female genitourinary tract may lead to initial surgical resection. In some of these patients, initial surgical resection may result in complete removal of malignancy. However, due to propensity for systemic spread, all such patients should be considered for combination chemotherapy after surgical resection.

The role of radiation therapy is not clear; however, prolonged survival has been reported in limited numbers of patients after radiation therapy alone when presenting at a very limited stage.

Brain metastases are uncommon in patients with extrapulmonary small cell carcinomas compared with small cell lung carcinoma, and prophylactic cranial irradiation is probably not necessary in most patients. In a study of 280 patients with extrapulmonary small cell carcinoma, 18 (6.4%) developed brain metastases. Median overall survival among these patients was 10.1 months. Eleven patients (61%) received cranial irradiation, 12 (67%) received palliative chemotherapy, and two (0.17%) received prophylactic cranial irradiation. [24]

Estimates of brain metastasis in the literature range from 1.7% up to 40%. Frequency varies by site. Esophageal small cell cancer has a low incidence of brain metastasis, as do genitourinary, colorectal, small bowel, and appendix small cell cancers, and prophylactic cranial irradiation is not recommended in these patients. The frequency of brain metastasis is much higher in prostate small cell carcinoma, with estimates ranging from 16% to 19%, and in head and neck small cell cancer, with a frequency of up to 41%. Prophylactic cranial irradiation should be considered in these patients. [25]


Surgical Care

See Medical Care.



The care of patients with limited extrapulmonary small cell carcinoma should involve a multidisciplinary approach that includes initial consultations with surgical, medical, and radiation oncologists to devise the most appropriate management plan.