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Klin Padiatr 2012; 224(03): 170-173
DOI: 10.1055/s-0032-1308987
Case Report
© Georg Thieme Verlag KG Stuttgart · New York

Malignant Peritoneal Mesothelioma in a 16-Year-Old Girl: Presentation of a Rare Disease

Malignes peritoneales Mesotheliom bei einem 16-jährigen Mädchen: Manifestation einer seltenen Erkrankung
I. B. Brecht
1    Pediatric Hematology and Oncology, University Hospital ­Erlangen, Germany
,
A. Agaimy
2    Institute of Pathology, University Hospital Erlangen, Germany
,
M. Besendörfer
3    Pediatric Surgery, University Hospital Erlangen, Germany
,
R. Carbon
3    Pediatric Surgery, University Hospital Erlangen, Germany
,
F. C. Thiel
4    Obstetrics and Gynecology, University Hospital Erlangen, Germany
,
O. Rompel
5   Pediatric Radiology, University Hospital Erlangen, Germany
,
D. Osinski
1    Pediatric Hematology and Oncology, University Hospital ­Erlangen, Germany
,
T. Langer
1    Pediatric Hematology and Oncology, University Hospital ­Erlangen, Germany
,
M. Metzler
1    Pediatric Hematology and Oncology, University Hospital ­Erlangen, Germany
,
W. Holter
1    Pediatric Hematology and Oncology, University Hospital ­Erlangen, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
18 April 2012 (online)

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Abstract

Background:

Malignant peritoneal mesothelioma is extremely rarely seen in young patients.

Patient:

A 16 year-old girl presented with appendicitis-like acute abdominal pain. Intra-operatively, multiple confluent peritoneal nodules were seen on the entire greater omentum and in the pelvis infiltrating the uterus and both ovaries. Biopsies were obtained and interpreted as serous ovarian carcinoma. Radical surgical resection and hyperthermic intraperitoneal chemotherapy ­(HIPEC) with carboplatin was performed and followed by 2 cycles of carboplatin/paclitaxel. Histological reevaluation showed characteristic features of epithelioid peritoneal mesothelioma and ruled out serous ovarian cancer. Therapy was continued with 6 cycles of pemetrexed/cisplatin.

Results:

3 months after end of chemotherapy vital tumor tissue was found in the recess behind the liver, which could be resected completely. The patient is currently disease-free 17 months after initial diagnosis.

Conclusion:

Malignant peritoneal mesothelioma in young female patients might be under-recognized and possibly misdiagnosed as ovarian serous carcinoma in some cases. International and interdisciplinary cooperation is necessary in order to provide evidence based guidelines for diagnosis and treatment in the future.

Zusammenfassung

Hintergrund:

Das maligne peritoneale Mesothe­liom wird bei jungen Patienten extrem selten beobachtet.

Patient:

Ein 16-jähriges Mädchen präsentierte sich mit appendizitisartigem akutem Bauchschmerz. Intraoperativ fanden sich konfluierende Tumormassen im gesamten Omentum und im Beckenperitoneum mit Infiltration beider Ova­rien und des Uterus. Biopsien wurden durchgeführt und diese als seröses Ovarialkarzinom interpretiert. Es wurde eine radikale chirurgische Resektion ­mit anschließender hyperthermer intraperitonealer Che­moperfusion (HIPEC) mit Carboplatin durchgeführt, gefolgt von 2 Blöcken Carboplatin/Paclitaxel. Eine histopathologische Reevaluation zeigte den cha­rakteristischen Befund eines epitheloiden malignen peritonealen Mesothelioms. Ein serö­ses Ovarialkarzinom konn­te ausgeschlossen werden. Die Therapie wurde fortgeführt mit 6 Zyklen Pemetrexed/Cisplatin.

Ergebnisse:

3 Monate nach Ende der Chemotherapie wurde vitales Tumorgewebe im Recessus hinter der Leber gefunden, welches komplett reseziert werden konnte. Die Patientin lebt derzeit rezidivfrei (17 Monate nach Primärdiagnose).

Schlussfolgerung:

Das maligne peritoneale Me­sotheliom ist bei jungen Patientinnen möglicherweise unterdiagnostiziert, einige Fälle könn­ten als seröse Karzinome fehlinterpretiert werden. Internationale und interdisziplinäre Ko­operation ist notwendig, um in Zukunft evidenzbasierte Leitlinien für Diagnose und Behandlung erstellen zu können.

Key words

malignant peritoneal mesothelioma - rare tumors - pediatric - ovarian carcinoma

Schlüsselwörter

malignes peritoneales Mesotheliom - seltene Tumoren - pädiatrisch - Ovarialkarzinom
 

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