Two autopsy cases of desmoplastic small round cell tumor

Desmoplastic small round cell tumor (DSRCT) is a rare aggressive malignant tumor. It is a refractory tumor and the median overall survival is very short. We report two autopsy cases of DSRCT, both of which were already advanced and metastasized at the first medical examination. Both cases showed typ...

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Bibliographic Details
Published in:Pathology international 2020-03, Vol.70 (3), p.171-178
Main Authors: Yamamoto, Yohei, Nishijima‐Matsunobu, Aki, Hiroshima, Yuko, Enomoto, Katsuhiko, Inoue, Masahiro, Muto, Osamu, Suzuki, Maya, Nanjo, Hiroshi, Shibata, Hiroyuki, Omori, Yasufumi
Format: Article
Language:English
Subjects:
Adult
Autopsies
Autopsy
Chemotherapy
chromosome translocation
Computed tomography
desmoplastic small round cell tumor
Desmoplastic Small Round Cell Tumor - diagnostic imaging
Desmoplastic Small Round Cell Tumor - drug therapy
Desmoplastic Small Round Cell Tumor - genetics
Desmoplastic Small Round Cell Tumor - pathology
EWSR1‐WT1
fusion gene
Genetics
Histopathology
Humans
In Situ Hybridization, Fluorescence
Localization
Male
Oncogene Proteins, Fusion - genetics
Pancreatic cancer
Pancreatic carcinoma
Pancreatic Neoplasms
Pancreatic Neoplasms - diagnostic imaging
Pancreatic Neoplasms - drug therapy
Pancreatic Neoplasms - genetics
Pancreatic Neoplasms - pathology
Physical examinations
Signs and symptoms
Survival
Tomography
Tomography, X-Ray Computed
Translocation, Genetic - genetics
Tumors
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Description
Summary:Desmoplastic small round cell tumor (DSRCT) is a rare aggressive malignant tumor. It is a refractory tumor and the median overall survival is very short. We report two autopsy cases of DSRCT, both of which were already advanced and metastasized at the first medical examination. Both cases showed typical DSRCT findings in terms of localization of the lesions, histopathology and genetics, but the rate of disease progression was quite different. Survival after initial symptoms in Case 1 was only 12 months. On the other hand, survival after primary hospitalization in Case 2 was 42 months. The Case 2 patient initially received chemotherapy for advanced pancreatic carcinoma, because a nodule of the pancreatic tail was found on computed tomography (CT) scan. After chemotherapy, tumor regression was observed on CT scan. It is thus implied that adoption of the regimen for pancreatic carcinoma might have been one of reasons of the long survival in Case 2.
ISSN:1320-5463
1440-1827
DOI:10.1111/pin.12897