Extrapulmonary Small Cell Carcinoma Treatment & Management

Updated: Jun 11, 2021
  • Author: Irfan Maghfoor, MD; Chief Editor: Nagla Abdel Karim, MD, PhD  more...
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Approach Considerations

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

No randomized studies exist to guide decisions regarding management of EPSCC. 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. [10, 11] 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. [12]

Although prophylactic brain irradiation is recommended for small cell lung cancer because of the frequency of brain metastases, only 2 of the 41 EPSCC patients (4.9%) in an Israeli study had brain metastases. Those authors concluded that prophylactic brain irradiation should not be recommended in EPSCC. [13]

Tumor lysis syndrome can occur rapidly in patients with small cell carcinomas on institution of therapy.


Medical Care

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. 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. [14]

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. Patients 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 after radiation therapy alone has been reported in limited numbers of patients who presented with very-limited-stage disease.

Estimates of brain metastasis in the literature range from 1.7% up to 40%. [15, 16] 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. [17, 18]




Data linking extrapulmonary small cell carcinoma to tobacco smoking are inconclusive; however, at least one study has reported that most patients with extrapulmonary small cell carcinoma in the study were former or current smokers. Since smoking is implicated as an etiologic agent in several different kinds of malignancies, smoking cessation education and programs with a concerted efforts form health care providers and government agencies are needed.


Long-Term Monitoring

Patients with extrapulmonary small cell carcinoma require close monitoring for adverse effects of chemotherapy. Complete blood counts with differential counts should be checked prior to each cycle of chemotherapy to ensure adequate hematologic recovery. Liver and renal function should be monitored to detect toxicity from chemotherapy as well as to assess if adjustment of chemotherapy doses is needed.

Response to therapy is monitored by obtaining CT scans after 2 cycles of chemotherapy before chemotherapy is continued.

If elevated prior to therapy, serum lactate dehydrogenase is a good marker and should be monitored.