Journal
MEDICINE
Volume 100, Issue 12, Pages -Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MD.0000000000025264
Keywords
clear cell type; immunohistochemistry; primary peritoneal epithelioid mesothelioma; whole-exome sequencing
Categories
Funding
- Beijing Municipal Administration of Hospitals' Ascent Plan [DFL20180701]
Ask authors/readers for more resources
Primary peritoneal epithelioid mesothelioma of clear cell type is a rare entity that requires differentiation from various clear cell tumors. It is characterized by specific genetic alterations, highlighting the importance of individualized therapy guidance. Cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy helps achieve long-term survival.
Rationale: Primary peritoneal epithelioid mesothelioma of clear cell type is an extremely rare entity composed of clear cytoplasm. It is challenging to diagnose because of the morphological resemblance to clear cell tumor. Patient's concerns: A 69-year-old male patient had swollen lymph nodes in the right inguinal region for 7 months and was constipated for 1 month. Diagnosis: The patient was diagnosed as peritoneal epithelioid mesothelioma of clear cell type based on computed tomography scan, pathology, immunohistochemistry, special staining and whole-exome sequencing. This patient harbored VHL gene alteration in exon 1 and homologous recombination defect (with a score of 45). This finding indicated that this patient might be sensitive to platinum-based therapy and Poly ADP-ribose Polymerase (PARP) inhibitor. This patient carried no microsatellite instability, a low level of tumor mutation burden, and a high extent of intratumoral heterogeneity. Eighteen neoantigens were detected. Interventions: The patient received surgery-based multidisciplinary treatment by integrating cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). HIPEC was administered with docetaxel 120 mg plus cisplatin 120 mg, at 43 degrees C, for 60 minutes. After operation, the patient received intravenous (IV) chemotherapy with docetaxel 60 mg, pemetrexed 750 mg and cisplatin 100 mg, and then intraperitoneal (IP) chemotherapy with docetaxel 40 mg. The patient received interventional therapy of hepatic artery embolization for 5 times. Outcomes: Regular follow-up was performed until Oct 14, 2020. The patient died 31.6 months later owing to incomplete intestinal obstruction. Lessons: Primary peritoneal epithelioid mesothelioma of clear cell type needs to be differentiated from a variety of clear cell tumors. This disease is characterized by specific genetic alteration. Whole-exome sequencing contributes to guide individualized therapy. CRS-HIPEC helps achieve long-term overall survival.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available