Article

Cotyledonoid Dissecting Leiomyoma of the Uterus: A Case Report and Review of the Literature

Department of Pathology, Yeungnam University College of Medicine, Daegu, Korea.
Journal of Korean Medical Science (Impact Factor: 1.25). 01/2003; 17(6):840-4. DOI: 10.3346/jkms.2002.17.6.840
Source: PubMed

ABSTRACT "Cotyledonoid dissecting leiomyoma" or "Sternberg tumor" is a very rare variant of smooth muscle tumors with a distinctive gross appearance. We describe a similar lesion, probably the sixth reported case, comparing its clinicopathological features with those of previous cases. A 26-yr-old nulliparous woman underwent laparotomy for a large pelvic mass replacing the postero-lateral aspect of the uterus with extension into the left pelvic cavity in the form of numerous exophytic congested small nodules. The tumor was removed by resection without hysterectomy after frozen section examination. Histologically, there were variable sized micronodules of benign smooth muscle fascicles, which were separated by fibrous connective tissue with a marked hydropic change and rich vascularity. Immunohistochemical and ultrastructural studies were helpful for confirmation of the smooth muscle nature, but not useful for the definitive diagnosis. Due to bizarre, sarcoma-like gross appearances, this type of lesion should be subjected to frozen section examination in order to avoid overtreatment and preserve the fertility in young women.

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    ABSTRACT: SUMMARY Introduction: Cotyledonoid dissecting leiomyoma or Sternberg tumor is a benign smooth muscle tumor with an unusual growth pattern. Patients and methods: Histopa- thological findings of two cases (41 and 56 year old fema- les) of this rare variant of uterine leiomyoma are analyzed. Results: In both cases several nodules conforming an ute- rus-depending great mass occupying the pelvis were found. Total hysterectomy, bilateral salpingo-oophorectomy and pelvic masses removal were performed. On microscopic examination, several bulbous masses with alarming appea- rance were found. Masses were formed by bundles of smo- oth muscle cells extending over the external uterine surface and continuing with an intramyometrial tumoral component dissecting the uterine wall. Significant nuclear atypia, mito- ses and necrosis were absent. Conclusions: In spite of the alarming aspect of these lesions, their evolution is benign in most cases. The importance of delivering a precise patholo- gical diagnosis will prevent an inadequate treatment of the patients. An intraoperative pathological examination is recommended. RESUMEN Introducción: El leiomioma cotiledonoide disecante del útero o tumor de Sternberg es un tumor benigno de mús- culo liso caracterizado por tener un patrón de crecimiento inusual. Pacientes y métodos: Presentamos los hallazgos histopatológicos de dos casos (pacientes de 41 y 56 años) de esta rara variante de leiomioma uterino. Inmunohistoquími- camente se confirmó la naturaleza muscular lisa de las lesiones. Resultados: En ambas pacientes se observó una gran masa polilobulada dependiente del útero que ocupaba la pelvis. Se realizó histerectomía y doble anexectomía con exéresis de masas pélvicas. En el estudio anátomopatológi- co se observaron múltiples masas bulbosas de aspecto alar- mante compuestas por haces de músculo liso que se exten- dían por la superficie externa del útero y se continuaban con un componente intramiometrial que disecaba la pared uteri- na. No se encontraron atipia nuclear significativa, mitosis ni necrosis. Conclusiones: A pesar de su aspecto alarmante estos tumores, su comportamiento es benigno, por lo que hacemos hincapié en la importancia de su adecuado diag- nóstico. Es muy aconsejable la realización de un estudio microscopico intraoperatorio para realizar un adecuado tra- tamiento de las pacientes.
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    ABSTRACT: We report a case of a rare, unusual benign tumor, the cotyledonoid leiomyoma or Sternberg tumor, detected incidentally in a 67-year-old woman who underwent total hysterectomy and bilateral salpingo-oophorectomy because of a persisting left ovarian cyst of 5 cm. This is the 14th case of cotyledonoid leiomyoma and 2nd case which had no intrauterine portion but had extrauterine extensions. At laparotomy, it was detected that a deep red nodular papillary tumor had been dissecting the posterior leaf of the left broad ligament, projecting into the abdominal cavity just near the left cornu of the uterus, and extending in downward direction. The remaining portion was lying between the layers of the left broad ligament and arose from the subserosa of the left side of the uterine isthmus. Although the bizarre and unusual shape was in favor of malignity, the frozen-section examination revealed a benign histology. Although the cotyledonoid leiomyoma is a benign entity, it may suggest a malignant disease owing to its unusual sarcomatoid appearance and its rarity. Therefore, awareness of obstetricians and gynecologists regarding this rare entity will prevent unnecessary anxiety and interventions.
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