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© 2003 American Society for Clinical Oncology
Small-Cell Cancers, and an Unusual Reaction to ChemotherapyCASE 1. EXTRAPULMONARY SMALL-CELL CARCINOMA ARISING IN THE PROSTATEDana-Farber Cancer Institute, Brigham & Women’s Hospital, and Harvard Medical School, Boston, MA
A 68-year-old man was noted to have a slowly increasing serum prostate-specific antigen (PSA) level during the 1990s. In 1998, his PSA reached 10.7, which triggered a prostate biopsy and the diagnosis of adenocarcinoma. He underwent a radical retropubic prostatectomy, which revealed Gleason 3 + 5 prostatic adenocarcinoma, with focal invasion of extraprostatic tissue but no evidence of seminal vesicle or lymph node involvement (T3aN0). His PSA became undetectable for more than 2 years before increasing to 0.1 in 2001. At that time he also suffered a fall, which led to progressive left groin pain. Pelvic computed tomography (CT) scan revealed a comminuted fracture of the left inferior pubic ramus (Fig 1A
Extrapulmonary small-cell carcinoma was first described in 1930 by Duguid and Kennedy3 and is estimated to occur in approximately 1,000 patients per year in the United States.4 Small-cell carcinoma arising from extrapulmonary sites represents 2% to 4% of all small-cell carcinoma. The most common sites of origin include gynecologic organs, particularly the cervix; the gastrointestinal tract, particularly esophagus and colon; the upper airway, sinuses, and salivary glands; and genitourinary organs, particularly the prostate.5 Paraneoplastic syndromes occur in approximately 10% of patients.6 In the largest single-institution series from the Mayo Clinic, approximately 75% of patients presented with localized disease and were treated with surgery or combined chemotherapy and radiation.7 However, 75% recurred at a median of 6 months, leading to an overall survival of 18 months for localized disease. The single best predictor of long-term disease-free survival after local therapy was disease localized to the primary organ at presentation, without any regional or lymph node involvement.7 For patients with widespread disease, platinum-based chemotherapy regimens led to a 73% response rate with a median duration of response of 8.5 months. In this series, 5-year overall survival for the entire population was 13%.7 Extrapulmonary small-cell carcinoma arising in the prostate has been described in several case series and case reports.6,8 The median age of the patients is approximately 65 years. Of note, 30% of patients presented initially with prostatic adenocarcinoma, similar to our patient, and recurred with small-cell carcinoma. Twenty percent of patients presented with combined adenocarcinoma and small-cell carcinoma, and 50% of patients presented with small-cell carcinoma.6,8 These patients did poorly, with a median survival of 5 months for patients presenting with small-cell carcinoma6 and 23 months for patients presenting initially with adenocarcinoma.6,8 Most of these patients were treated with antiandrogens, without evidence of clinical response. Because small-cell carcinomas of the prostate do not express androgen receptors,9 this is not surprising, and patients now receive therapy specific for small-cell carcinoma,10,11 with a 60% response rate and median survival of 9 to 10 months.10,11 The coexistence of adenocarcinoma with small-cell carcinoma in many of these tumors has raised the question of the cell type of origin. Many prostatic adenocarcinomas show areas of focal neuroendocrine differentiation, and many extrapulmonary small-cell carcinomas of the prostate are associated with an adenocarcinoma component.2,6,8,9 Some authors have therefore hypothesized that both tumor types arise from a pluripotent stem cell that is able to differentiate into both a mature prostatic epithelial cell and a neuroendocrine cell,2,9 but additional scientific investigation will be needed to confirm this hypothesis.
REFERENCES 1. Agoff SN, Lamps LW, Philip AT, et al: Thyroid transcription factor-1 is expressed in extrapulmonary small cell carcinomas but not in other extrapulmonary neuroendocrine tumors. Mod Pathol 13:238–242, 2000[CrossRef][Medline] 2. Ro JY, Tetu B, Ayala AG, et al: Small cell carcinoma of the prostate. Cancer 59:977–982, 1987[CrossRef][Medline] 3. Duguid JB, Kennedy AM: Oat-cell tumors of mediastinal glands. J Pathol Bacteriol 33:93–99, 1930[CrossRef] 4. Remick SC, Ruckdeschel JC: Extrapulmonary and pulmonary small cell carcinoma: tumor biology, therapy, and outcome. Med Pediatr Oncol 20:89–99, 1992[Medline] 5. Remick SC, Hafez GR, Carbone PP: Extrapulmonary small-cell carcinoma. Medicine 66:457–471, 1987[Medline] 6. Yu DS, Chang SY, Wang J, et al: Small cell carcinoma of the urinary tract. Br J Urol 66:590–595, 1990[Medline] 7. Galanis E, Frytak S, Lloyd RV: Extrapulmonary small cell carcinoma. Cancer 79:1729–1736, 1997[CrossRef][Medline] 8. Oesterling JE, Hauzeur CG, Farrow GM: Small cell anaplastic carcinoma of the prostate: A clinical, pathological and immunohistological study of 27 patients. J Urol 147:804–807, 1992[Medline] 9. Di Sant’Agnese PA: Neuroendocrine differentiation in prostatic carcinoma. Cancer 75:1850–1859, 1995 (suppl)[CrossRef]
10. Papandreou CN, Daliani DD, Thall PF, et al: Results of a phase II study with doxorubicin, etoposide and cisplatin in patients with fully characterized small cell carcinoma of the prostate. J Clin Oncol 20:3072–3080, 2002 11. Amato RJ, Logothetis CJ, Hallinan R, et al: Chemotherapy for small cell carcinoma of prostatic origin. J Urol 147:935–937, 1992[Medline]
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Copyright © 2003 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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