We describe a novel EWSR1-HOXB13-fusion in a fibroblastic tumor from the abdominal wall of a 29-year-old woman. This tumor caused intermittent intense pain and had grown to approximately 5 cm in size over two years. The tumor was located beneath subfascial section of the abdominal wall and was invading the abdominal cavity and pressing on the liver. The tumor was well-circumscribed and consisted of intersected fascicles of monomorphic spindle-shaped cells with uniform ovoid nuclei lacking nuclear pleomorphism or mitotic activity. This tumor was immunohistochemically negative for pan-cytokeratin AE1/AE3, desmin, SMA, S100, myogenin, MyoD1, CD34, melanosome, SOX10, STAT6, SS18-SSX, and ERG. H3K27me3 was retained. RNA sequencing revealed a unique EWSR1-HOXB13-fusion, and strong, diffuse nuclear immunostaining for HOXB13 was observed. No local recurrence or evident distant metastasis were observed over eight months without chemotherapy, implying that the behavior of this tumor is not yet known.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1007/s00428-022-03282-y | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Multinucleate cell angiohistiocytoma: a case series and literature review.
Pol J Pathol
January 2025
Department of Dermatology, Medical University of Warsaw, Warsaw, Poland.
Multinucleate cell angiohistiocytoma (MCAH) is a rare benign cutaneous entity. It classically presents as slowly progressive erythematous to violaceous papules on the distal extremities of middle-aged or elderly women. The entity may clinically resemble granuloma annulare, lichen planus, and several cutaneous vascular proliferations.
View Article and Find Full Text PDFRapid growth of a solitary fibrous tumor of the pleura after slow long-term progression: a case report.
J Cardiothorac Surg
January 2025
Division of Thoracic Surgery, Department of Surgery, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, 602-8566, Kyoto, Japan.
Background: Solitary fibrous tumors (SFTs) of the pleura are usually benign. We present a case of SFT of the pleura which grew rapidly after slow long-term progression.
Case Presentation: A 78-year-old man was referred to our hospital for left-sided back pain and shortness of breath.
Three-dimensional (3D)-printed custom-made titanium ribs for chest wall reconstruction post-desmoid fibromatosis resection.
Comput Assist Surg (Abingdon)
December 2025
Department of Thoracic Surgery, The First People's Hospital of Changzhou, The Third Affiliated Hospital of Soochow University, Changzhou, China.
Desmoid fibromatosis (DF) is a rare low-grade benign myofibroblastic neoplasm that originates from fascia and muscle striae. For giant chest wall DF, surgical resection offer a radical form of treatment and the causing defects usually need repair and reconstruction, which can restore the structural integrity and rigidity of the thoracic cage. The past decade witnessed rapid advances in the application of various prosthetic material in thoracic surgery.
View Article and Find Full Text PDFPleural Rosai-Dorfman disease complicated with renal clear cell carcinoma: a case report and literature review.
Front Oncol
January 2025
Department of Radiology, Daping Hospital, Army Medical University, Chongqing, China.
Background: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-malignant disorder characterized by excessive proliferation of histiocytes, the cause of which remains unknown. Although the lymph nodes are the most commonly affected site, some patients may present with extranodal involvement, particularly in the skin, nasal cavity, eyes, and bones. In this report, we aim to present a unique case of RDD with pleural involvement in a 61-year-old patient.
View Article and Find Full Text PDFAn elderly patient with low-grade fibromyxoid sarcoma with early postoperative recurrences and metastases: a case report.
J Med Case Rep
January 2025
Division of Orthopedic Surgery, Hillel Yaffe Medical Center, Ha-Shalom St, 3820302, Hadera, Israel.
Background: Low-grade fibromyxoid sarcoma is a rare soft tissue tumor characterized by a benign histological appearance but with a high potential for recurrence and metastasis. First described by Evans in 1987, recurrence and metastasis can occur decades after the initial diagnosis, complicating long-term management.
Case Presentation: We report the case of an 83-year-old Jewish female patient diagnosed with low-grade fibromyxoid sarcoma in her right shoulder.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!