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

Abstract

Malignant peritoneal mesothelioma is extremely rarely seen in young patients.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.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.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.

Multicystic and diffuse malignant peritoneal mesothelioma in children

“…There is a considerable lack of data regarding the best treatment strategy in the pediatric population. The use of CRS‐HIPEC for the treatment of DMPM was reported before in only 13 pediatric cases, with favorable outcomes 3,5,33,37 . Our study confirms that CRS‐HIPEC could be used as first‐line treatment in children with resectable disease who are fit for major surgery 37‐39 .…”

“…Surgical biopsy for immunohistological analysis is a key exam for DMPM diagnosis. The International Mesothelioma Interest Group recommends analysis of calretinin, cytokeratins 5/6, WT1, and D2‐40 as positive mesothelial markers, and ACE and MOC‐31 as positive carcinoma markers to differentiate malignant mesotheliomas from adenocarcinoma, ovarian carcinoma, or renal carcinoma 5,23‐25 . Churg et al.…”

“…Only a few pediatric cases of DMPM are reported in literature. [3][4][5][6][7][8][9][10][11] Its incidence is estimated to be 0.5-1 case/10 million/year. 12 The female-to-male ratio is 2:1.…”

“…The initial symptoms of DMPM are unspecific, such as abdominal pain, abdominal distension, ascitis and weight loss. Only a few pediatric cases of DMPM are reported in literature 3‐11 . Its incidence is estimated to be 0.5‐1 case/10 million/year 12 .…”

Primary Ovarian Mesothelioma: A Case Series with Electron Microscopy Examination and Review of the Literature

“…Several cases of secondary involvement of the ovary by diffuse malignant peritoneal mesothelioma have been reported in the literature; more rarely, ovarian primary malignant mesothelioma may involve the peritoneum [ 81 , 82 ]. Indeed, clinical and morphological distinction between some entities (e.g., well-differentiated peritoneal mesothelioma or florid peritoneal mesothelial proliferations) may be difficult due to some overlapping morphological and immunohistochemical (IHC) features, including papillary structures, clinical symptoms and imaging findings [ 96 , 97 , 98 , 99 , 100 , 101 , 102 , 103 , 104 , 105 , 106 , 107 , 108 , 109 , 110 , 111 , 112 , 113 ]. This is mainly true if we also consider the new knowledge on the emerging entity of the mesothelioma in situ that can remain hidden for a long time [ 114 , 115 ].…”