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. 2012 Dec;72(12):1132-1136.
doi: 10.1055/s-0032-1328076.

18-Year-Old Woman with an Embryonal Rhabdomyosarcoma of the Uterus in Statu Nascendi

O Strahl  1 , A Hartmann  2 , F C Thiel  1 , M W Beckmann  3 , M P Lux  1
Affiliations

18-Year-Old Woman with an Embryonal Rhabdomyosarcoma of the Uterus in Statu Nascendi

O Strahl et al. Geburtshilfe Frauenheilkd. 2012 Dec.

Abstract
in English, German

Background: We report a case of an 18-year-old woman with an embryonal rhabdomyosarcoma in statu nascendi. Case: A fist-sized embryonal rhabdomyosarcoma of the uterus filling the vaginal vault was diagnosed in an adolescent with virgo intacta suffering from therapy resistant vaginal discharge, bleeding and bulging mass for six months. Further imaging revealed one suspicious pelvine lymph node. Excision of the tumour including the intracervical stalk was performed and followed by systemic multiagent chemotherapy. PET-CT scan presented a complete response after the third cycle. Histological complete response was shown by laparoscopic dissection of regional pelvic lymph nodes and curettage. Conclusion: Uterine Rhabdomyosarcoma should be considered as differential diagnosis of therapy resistant vaginal flour and bleeding in young women. Fertility-sparing therapy is possible in selected exceptional cases.

Hintergrund: Wir berichten über eine 18-jährige Patientin mit Erstdiagnose eines embryonalen Rhabdomyosarkoms des Uterus in statu nascendi. Fallbericht: Bei einer 18-jährigen Patientin G0, virgo intacta fiel ein übelriechender, zerfallender Tumor im Bereich der Scheide auf. Die durchgeführte Biopsie zeigte einen malignen mesenchymalen Tumor mit Verdacht auf embryonales Rhabdomyosarkom. Bei starker vaginaler Blutung wurde am Aufnahmetag ein MRT des Beckens durchgeführt. Hier wurde ebenfalls der Verdacht auf ein Leiomyosarkom ausgehend von der Vagina mit Zervixbeteiligung und zusätzlich eines metastasensuspekten Lymphknoten rechts pelvin diagnostiziert. Nach vaginaler Partialexzision des Primarius wurde mit systemischer Chemotherapie begonnen. Die Reevaluation mittels PET-CT nach dem 3. Zyklus der systemischen Therapie zeigte eine ycT0-ycN0-cM0-Situation. Die Komplettremission konnte durch laparoskopisch durchgeführte pelvine Lymphnodektomie rechts und fraktionierte Kürettage histologisch bestätigt werden. Fazit: Bei den jungen Patientinnen mit therapieresistenten vaginalen Fluor und Blutungen sollte bei der Differenzialdiagnose an ein uterines Rhabdomyosarkom gedacht werden. In Ausnahmefällen kann die Tumortherapie auch fertilitätserhaltend durchgeführt werden.

Keywords: embryonal rhabdomyosarcoma; sarcoma botryoides; uterine sarcoma; uterine tumor.

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Conflict of interest statement

Conflict of Interest None.

Figures

Fig. 1 a
Fig. 1 a
to f a–c Histopathological examination. d–f Immunhistochemical staining: d MyoD1; e myogenin; f desmin.
Fig. 2 a
Fig. 2 a
to f a, b MRI T2w before surgery: a intravaginal part of the tumour; b cavum uteri including tumour stalk. c–f 18F-FDG-PET/CT (after surgery): c, e residual tumour (arrow); d, f suspicious lymph node (arrow) and stimulated ovary (arrow head).
Fig. 3 a
Fig. 3 a
to d Intraoperative pictures final surgery after cycle 4. a Fundus uteri: Laparoscopic view from abdominal; b fundus uteri: hysteroscopic view cavum uteri; c, d ostium of fallopian tubes bilateral: hysteroscopic view cavum uteri.
Fig. 4
Fig. 4
CT thorax/abdomen (2012-04-10). No organ or lymph node metastases.
See this image and copyright information in PMC

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