Abstract
Background and Purpose:
Extrapulmonary small cell carcinoma (EPSCC) is a rare disease, which has a slightly better prognosis than small cell lung cancer, but still dismal. Gynecologic small cell malignancies tend to show a better survival than similar histologies of other regions. However, of five reported cases of vulvar manifestation only one patient was disease-free at the time of publication with limited follow-up.
Case Report:
The authors describe a case of locally advanced small cell vulva carcinoma infiltrating the anal sphincter and urethra with spread to inguinal lymph nodes treated by radiochemotherapy and regional hyperthermia. After three cycles of carboplatin/ etoposide, computed tomography and magnetic resonance imaging indicated only little regressive transformations but overall stable disease. Surgical options were excluded. Therefore, curative radiotherapy to a total dose of > 65 Gy to macroscopic tumor, chemotherapy with cisplatin weekly, and regional hyperthermia were performed. Acute severe toxicity was limited to skin reactions. Despite the disadvantageous situation with inguinal lymph node metastases and chemoresistance, the multimodal therapy yielded a 5-year disease-free survival.
Conclusion:
Thus, the trimodal regimen of radiochemotherapy plus regional hyperthermia offered a curative chance in spite of resistance to the standard chemotherapy for irresectable, locally advanced small cell carcinoma of the vulva. Therefore, this approach merits further evaluation for limited disease of EPSCC.
Zusammenfassung
Hintergrund und Ziel:
Extrapulmonale kleinzellige Karzinome (EPSCC) sind eine seltene Tumorentität, die eine etwas bessere Prognose als kleinzellige Bronchialkarzinome besitzt. Innerhalb dieser Entität zeigen gynäkologische Tumoren ein etwas besseres Überleben. Allerdings war von den fünf beschriebenen Fällen kleinzelliger Vulvakarzinome zum Zeitpunkt der Auswertung nur eine Patientin mit kurzer Nachsorge tumorfrei.
Fallbericht:
Die Autoren beschreiben den Fall einer Patientin mit lokal fortgeschrittenem kleinzelligen Vulvakarzinom, welches den Sphincter ani, die Urethra und die inguinalen Lymphknoten involvierte. Nach der initialen Chemotherapie mit Carboplatin/ Etoposid zeigten sich leichte regressive Veränderungen, aber messtechnisch keine relevante Größenabnahme im Sinne einer „stabilen Erkrankung“. Operative Behandlungsmöglichkeiten bestanden nicht. Daher wurde als kurative Modalität eine definitive Radiochemotherapie mit > 65 Gy auf den makroskopischen Tumor mit simultaner wöchentlicher Cisplatingabe plus lokoregionaler Tiefenhyperthermie durchgeführt (Abbildung 1). Höhergradige Akuttoxizitäten beschränkten sich auf die Haut. Trotz der ungünstigen Ausgangssituation mit lymphogener Metastasierung und fehlendem Ansprechen auf die initiale Chemotherapie konnte mit der multimodalen Therapie ein krankheitsfreies Überleben von 5 Jahren erreicht werden (Abbildung 2).
Schlussfolgerung:
Die trimodale Therapie mit Radiotherapie, Chemotherapie und Hyperthermie eröffnete trotz der initialen Resistenz gegenüber der Standardchemotherapie einen kurativen Ansatz bei einem inoperablen, lokal fortgeschrittenen kleinzelligen Vulvakarzinom. Dieses Ergebnis kann einen Ausgangspunkt für die Diskussion der Therapieoptionen von lokal begrenzten EPSCC bilden.
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Eckert, F., Fehm, T., Bamberg, M. et al. Small Cell Carcinoma of Vulva. Strahlenther Onkol 186, 521–524 (2010). https://doi.org/10.1007/s00066-010-2160-z
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DOI: https://doi.org/10.1007/s00066-010-2160-z