Journal of Pathology and Translational Medicine

Case Studies

WHO Grade IV Gliofibroma: A Grading Label Denoting Malignancy for an Otherwise Commonly Misinterpreted Neoplasm: Paola A. Escalante Abril, Miguel Fdo. Salazar, Nubia L. López García, Mónica N. Madrazo Moya, Yadir U. Zamora Guerra, Yadira Gandhi Mata Mendoza, Erick Gómez Apo, Laura G. Chávez Macías; J Pathol Transl Med. 2015;49(4):325-330. Published online June 17, 2015; DOI: https://doi.org/10.4132/jptm.2015.05.20; Correction in: J Pathol Transl Med 2015;49(6):538

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We report a 50-year-old woman with no relevant clinical history who presented with headache and loss of memory. Magnetic resonance imaging showed a left parieto-temporal mass with annular enhancement after contrast media administration, rendering a radiological diagnosis of high-grade astrocytic neoplasm. Tumour sampling was performed but the patient ultimately died as a result of disease. Microscopically, the lesion had areas of glioblastoma mixed with a benign mesenchymal constituent; the former showed hypercellularity, endothelial proliferation, high mitotic activity and necrosis, while the latter showed fascicles of long spindle cells surrounded by collagen and reticulin fibers. With approximately 40 previously reported cases, gliofibroma is a rare neoplasm defined as either glio-desmoplastic or glial/benign mesenchymal. As shown in our case, its prognosis is apparently determined by the degree of anaplasia of the glial component.

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Rare Pediatric Invasive Gliofibroma Has BRAFV600E Mutation and Transiently Responds to Targeted Therapy Before Progressive Clonal Evolution
Kristiyana Kaneva, Kee Kiat Yeo, Debra Hawes, Jianling Ji, Jaclyn A. Biegel, Marvin D. Nelson, Stefan Bluml, Mark D. Krieger, Anat Erdreich-Epstein
JCO Precision Oncology.2019; (3): 1. CrossRef

ThinPrep Cytological Findings of Desmoplastic Small Round Cell Tumor with Extensive Glandular Differentiation: A Case Study: Hyun-Jung Kim, Byeong Seok Sohn, Ji-Eun Kwon, Jeong Yeon Kim, Kyeongmee Park; Korean J Pathol. 2013;47(2):182-187. Published online April 24, 2013; DOI: https://doi.org/10.4132/KoreanJPathol.2013.47.2.182

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Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive neoplasm. The cytological diagnosis of this tumor has only been reported in a few cases. In most of these cases, the diagnosis was made using fine-needle aspiration cytology. Most DSRCTs resemble disseminated carcinomatoses in their clinical manifestation as well as cytomorphologically, even in young-adult patients. These authors report a case of using peritoneal-washing and pleural-effusion ThinPrep cytology to diagnose DSRCT, with extensive glandular differentiation and mucin vacuoles. We found that fibrillary stromal fragment, clinical setting, and adjunctive immunocytochemical staining were most helpful for avoiding misdiagnosis.

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Desmoplastic Small Round Cell Tumor Involving Serous Fluid: Cytologic Features and Diagnostic Pitfalls: A Series of 8 Cases
Nibras L Fakhri, Qiong Gan
American Journal of Clinical Pathology.2023; 160(4): 417. CrossRef
A Review of Effusion Cytomorphology of Small Round Cell Tumors
Lucy M. Han, Christopher J. VandenBussche, Mads Abildtrup, Ashish Chandra, Poonam Vohra
Acta Cytologica.2022; 66(4): 336. CrossRef
Intra-abdominal desmoplastic small blue round cell tumor: A case report
Tareq Hamed Al Taei, Hasan Al Fardan, Sarah Ali Al Mail
Radiology Case Reports.2022; 17(12): 4502. CrossRef
Desmoplastic Small Round Cell Tumor of the Kidney: Report of a Case, Literature Review, and Comprehensive Discussion of the Distinctive Morphologic, Immunohistochemical, and Molecular Features in the Differential Diagnosis of Small Round Cell Tumors Affec
Carlos A. Galliani, Michele Bisceglia, Antonio Del Giudice, Giuseppe Cretì
Advances in Anatomic Pathology.2020; 27(6): 408. CrossRef
Intra-abdominal desmoplastic small round cell tumors: CT and FDG-PET/CT findings with histopathological association
JINGJING CHEN, ZENGJIE WU, BINBIN SUN, DACHENG LI, ZHENGUANG WANG, FANGJUN LIU, HUI HUA
Oncology Letters.2016; 11(5): 3298. CrossRef
Desmoplastic small round cell tumor with sphere‐like clusters mimicking adenocarcinoma
Yukinori Hattori, Akihiko Yoshida, Naoshi Sasaki, Yasuo Shibuki, Kenji Tamura, Koji Tsuta
Diagnostic Cytopathology.2015; 43(3): 214. CrossRef
Tumor intraabdominal desmoplásico de células pequeñas y redondas
Andrés Alejandro Briseño-Hernández, Deissy Roxana Quezada-López, Lilia Edith Corona-Cobián, Agar Castañeda-Chávez, Alfonso Tonatiuh Duarte-Ojeda, Michel Dassaejv Macías-Amezcua
Cirugía y Cirujanos.2015; 83(3): 243. CrossRef
Intra-abdominal desmoplastic small round cell tumour
Andrés Alejandro Briseño-Hernández, Deissy Roxana Quezada-López, Lilia Edith Corona-Cobián, Agar Castañeda-Chávez, Alfonso Tonatiuh Duarte-Ojeda, Michel Dassaejv Macías-Amezcua
Cirugía y Cirujanos (English Edition).2015; 83(3): 243. CrossRef
Diagnostic Pitfalls of Differentiating Desmoplastic Small Round Cell Tumor (DSRCT) From Wilms Tumor (WT)
Michael A. Arnold, Lynn Schoenfield, Berkeley N. Limketkai, Christina A. Arnold
American Journal of Surgical Pathology.2014; 38(9): 1220. CrossRef

Case Reports

The Cytologic Features of Desmoplastic Small Round Cell Tumor with Intranuclear Inclusions : A Case Report .: Ho Chang Lee, Hye Suk Han, Ok Jun Lee; Korean J Pathol. 2009;43(3):279-284.; DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.3.279

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Abstract PDF: Desmoplastic small round cell tumor (DSRCT) is a rare neoplasm of young adults and it is characterized by polyphenotypic differentiation. We experienced a case of abdominal DSRCT that occurred in a 19-year-old female who presented with painful swelling of her right forearm. The tumor was cytokeratin-negative and it exhibited some tumor cells with intranuclear inclusions. Molecular demonstration of EWS-WT1 fusion transcripts is particularly useful to confirm the diagnosis of DSRCT without epithelial differentiation. We report here on a case of cytokeratin-negative DSRCT that showed an unusual feature of intranuclear inclusions.

Desmoplastic Small Round Cell Tumor with Ovarian Involvement: A Case Report.: Sang Hwa Lee, Wan Seop Kim, Ji Hoon Kim, Hye Seung Han, So Dug Lim, Sang Yoon Kim, Tae Sook Hwang; Korean J Pathol. 2009;43(2):185-188.; DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.2.185

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Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm that preferentially involves the abdominal and pelvic cavities in relatively young males. We present a rare case of DSRCT arising in the ovary of a 16-year-old girl. During surgery, a 15 cm-sized huge mass was noted in the right ovary and wide spreading of the tumor was identified in the left ovary, uterine wall, and omentum and bowel wall. Histological investigation showed nests of small round cells with round nuclei and scanty eosinophilic cytoplasm accompanied with dense desmoplastic stroma. The immunohistochemistry showed that the tumor coexpressed epithelial, mesenchymal, and neuronal markers. The tumor cells ultrastructurally showed poorly developed cell junctions and occasionally showed intracytoplasmic aggregates of intermediate filaments. Molecular analysis of the tumor revealed chromosomal translocation t(11:22)(p13;q12) associated with the EWS-WT1 fusion protein. DSRCT should be included in the differential diagnosis of ovarian neoplasms in young patients.

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Desmoplastic small round cell tumor of the ovary
Ravi Hari Phulware, Maitrayee Roy, Neeta Singh, Sunesh Kumar, Sandeep R. Mathur
Indian Journal of Pathology and Microbiology.2021; 64(1): 206. CrossRef

Intraabdominal Desmoplastic Small Cell Tumors with Divergent Differentiation: Report of two cases with immunohistochemical and ultrastructural studies.: Young Ha Oh, Nam Hoon Kim, Joo Seob Keum, Moon Hyang Park; Korean J Pathol. 1996;30(1):40-49.

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Abstract PDF: We studied two intraabdominal desmoplastic small cell tumors. The patients were two men, 37 and 23 years old, with jaundice and palpable abdominal masses. On exploratory laparotomy, each patient revealed a huge mass in the greater omentum with disseminated peritoneal seeding, measuring 32 cm and 11 cm in its greatest dimension, respectively. The tumor involved the diaphragm, rectal shelf, and cul de sac in case 1, and it involved the porta hepatis, retroperitoneum, and serosal surface of the ascending and transverse colon in case 2. Omentectomy of the huge mass and satellite masses was performed in each patient. Both tumors showed nearly the same histopathologic features. The histologic pattern was suggestive of a metastatic small cell carcinoma, but there was no specific, single primary site. The tumors consisted of variably sized, discrete islands of epithelial-like small cells in dense desmoplastic stroma. The tumor cells revealed divergent epithelial, mesenchymal, and neural differentiation by histologic, immunohistochemical, and electron microscopic observations. Only one cycle of chemotherapy including cisplatin and VP-16 was given in case 1 because of a subsequent hepatic problem, who, thereafter, showed massive intraabdominal recurrent tumors 6 months after diagnosis. In case 2, the poor condition of the patient had made chemotherapy and radiotherapy impossible. Case 2 died of disseminated intravascular coagulation following progressive cachexia 7 months after diagnosis.

Desmoplastic Trichoepithelioma: A case report.: Jean A Kim, Young Sill Kim, Seok Jin Kang, Sun Moo Kim; Korean J Pathol. 1996;30(9):865-867.

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Abstract PDF: Desmoplastic trichoepithelioma is a solitary tumor of the hair follicle with female preponderance, most commonly located on the face. Clinically, the lesions appear annular and firm with a raised border and a depressed nonulcerated center, but the appearance is not so distinctive to hair follicle tumors that definite diagnosis is established by the histopathologic findings. We report a case of desmoplastic trichoepithelioma in a 67-year-old woman who had an asymptomatic, hard, annular lesion measuring 0.8 cm in diameter, with a raised border and a depressed center on the right cheek. Histopathologic findings showed narrow strands of basaloid cells, horny cysts, and desmoplastic stroma, with the features corresponding to the desmoplastic trichoepithelioma.

Imprint Cytology of a Desmoplastic Small Round Cell Tumor: A Case Report.: Joon Hyuk Choi, Yong Jin Kim, Jae Hwang Kim; Korean J Cytopathol. 2007;18(1):81-86.

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Abstract PDF: Desmoplastic small round cell tumor (DSRCT) is a rare malignant mesenchymal neoplasm. It mainly involves the abdominal or pelvic peritoneum of male adolescents. We report here the imprint cytologic features of a case of DSRCT occurring in the intraabdominal cavity of a 21-year-old man. A microscopic examination showed moderate cellularity. The tumor cells were singly arranged and arranged in clusters. The cells had round to oval nuclei with finely granular chromatin, inconspicuous nucleoli and scanty cytoplasm. Some tumor cells showed nuclear molding, and some cells had an epitheloid appearance with a large amount of lightly eosinophilic cytoplasm. A rosette-like pattern was present. Spindle-shaped, fibroblastic stromal cells were occasionally found. The tumor cells were immunoreactive for the markers cytokeratin (AE1/AE3), epithelial membrane antigen (EMA), desmin, vimentin and neuron specific enolase (NSE).

Primary Mucosal Desmoplastic Melanoma of Gingiva: A Case Report.: Gawon Choi, Jeong Won Kim, Soon Yuhl Nam, Kyung Ja Cho; Korean J Pathol. 2006;40(6):456-460.

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Abstract PDF: Desmoplastic melanoma (DM) is a rare malignant melanoma variant. Although DM mainly affects the skin chronically exposed to the sun, a small number of mucosal DM have been reported. Primary mucosal DM is difficult to diagnose because of its rarity and atypical histopathologic features. Here, we report a case of DM in a 52 year-old female who presented with a right cervical mass and upper gingival pigmentation. A CT scan revealed an ill-defined infiltrative mass 2 cm in size under the pigmented mucosa. She subsequently underwent a partial maxillectomy with neck dissection. Gross examination revealed that the mass exhibited a grayish white fibrotic cut surface and that the maxillary bone had been destroyed. Microscopically, the main mass was composed of cigar-shaped or wavy spindle cells with desmoplastic stroma under the melanoma in situ. The diagnosis of DM was confirmed immunohistochemically with S100 protein positivity and HMB45 negativity. The patient has survived for 29 months after the operation with a presumed metastatic focus.

A Case of Desmoplastic Fibroma of the Mandible.: Dong Won Kim, Tae Jung Kwon, Dong Wha Lee; Korean J Pathol. 1988;22(3):340-347.

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Abstract PDF: A case of desmoplastic fibroma of mandible in a 18 years old woman is presented. She had complained progressive swelling of right mandible for 4 years. Radiographically, a multilocular radiolucent of right hemimandibulectomy showed multinodular external surface without cortical destruction. Cut surfaces revealed grayish white, fibrous homogeneous appearance with firm consistency, sparing head portion. The maximum diameter of the tumor was 13 cm. Microscopically, the tumor was composed of interlacing bundles of monomorphic spindle-shaped cells with abundant intercellular collagen. Ultrastructurally, most tumor cells were fibroblastic-like cells with abundant RER and cytoplasmic fibrils, but a few disclosed transition to myofibroblasts. However, no fully developed myofibroblasts were seen.

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