Dedifferentiated soft tissue leiomyosarcoma with heterologous osteosarcoma component: case report and review of the literature

Clinical Sarcoma Research - 2020
Raffaele Gaeta1, Davide Matera2, Francesco Muratori2, Giuliana Roselli3, Giacomo Giulio Baldi4, Domenico Andrea Campanacci2, Alessandro Franchi1
1Department of Translational Research and of New Technologies in Medicine and Surgery, University of Pisa, Pisa, Italy
2Department of Orthopaedic Oncology and Reconstructive Surgery, Azienda Ospedaliera Universitaria Careggi, Florence, Italy
3Department of Diagnostic Imaging, Azienda Ospedaliera Universitaria Careggi, Florence, Italy
4Department of Oncology, “S. Stefano” Hospital, Prato, Italy

Tóm tắt

Abstract Background Soft tissue dedifferentiated leiomyosarcoma with heterologous osteosarcomatous component is an extremely rare entity described in only few cases in the literature. Case presentation We report the case of a 65-year-old male patient who, after initial inadequate surgery of a tumor of the left forearm, developed local recurrence that was treated with neoadjuvant chemotherapy, surgery and postoperative radiation therapy. Histologically the tumor showed an abrupt separation of two different patterns. One component consisted of interlacing fascicles of spindle cells with cigar-shaped nuclei strongly positive for smooth muscle actin, desmin and H-caldesmon. The other component consisted of a high-grade pleomorphic sarcoma with osteoid and chondroid matrix production, which positive for SATB2. Thus, a final diagnosis of dedifferentiated leiomyosarcoma was rendered. Fifteen months after treatment, the patient presented further local and distant relapse with pulmonary metastases and died 23 months after the first presentation. Discussion and conclusions Dedifferentiated leiomyosarcoma is a highly malignant neoplasm with a poor outcome. Extensive sampling of soft tissue leiomyosarcomas is recommended to detect possible dedifferentiated areas as they represent a crucial prognostic parameter.

Từ khóa


Tài liệu tham khảo

Lazar A, Evans HL, Shipley J. Leiomyosarcoma. In: Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F, editors. WHO classification of tumours of soft tissue and bone. 4th ed. Lyon: IARC Press; 2013.

Hashimoto H, Tsuneyoshi M, Enjoji M. Malignant smooth muscle tumors of the retroperitoneum and mesentery: a clinicopathologic analysis of 44 cases. J Surg Oncol. 1985;28:177–86.

Massi D, Beltrami G, Mela MM, Pertici M, Capanna R, Franchi A. Prognostic factors in soft tissue leiomyosarcoma of the extremities: a retrospective analysis of 42 cases. Eur J Surg Oncol. 2004;30:565–72.

Agaimy A, Semrau S, Koch M, Thompson LDR. Sinonasal leiomyosarcoma: clinicopathological analysis of nine cases with emphasis on common association with other malignancies and late distant metastasis. Head Neck Pathol. 2018;12:463–70.

Wang GY, Lucas DR. Primary leiomyosarcoma of bone: review and update. Arch Pathol Lab Med. 2019;143:1332–7.

Schmookler BM, Lauer DH. Retroperitoneal leyomiosarcoma. A clinicopathologic analysis of 36 cases. Am J Surg Pathol. 1983;7:269–80.

Guillou L, Coindre JM, Bonichon F, Nguyen BB, Terrier P, Collin F, Vilain MO, Mandard AM, Le Doussal V, Leroux A, Jacquemier J, Duplay H, Sastre-Garau X, Costa J. Comparative study of the National Cancer Institute and French Federation of Cancer Centers Sarcoma Group grading systems in a population of 410 adult patients with soft tissue sarcoma. J Clin Oncol. 1997;15:350–62.

Chen E, O’Connell F, Fletcher CD. Dedifferentiated leiomyosarcoma: clinicopathological analysis of 18 cases. Histopathology. 2011;59:1135–43.

Mentzel T, Fletcher CDM. Malignant mesenchymomas of soft tissue associated with numerous osteoclast-like giant cells mimicking the so-called giant cell variant of “malignant fibrous histiocytoma”. Virchows Arch. 1994;424:539–45.

Grabellus F, Sheu SY, Schmidt B, Flasshove M, Kuhnen C, Ruchholtz S, Taeger G, Schmid KW. Recurrent high-grade leiomyosarcoma with heterologous osteosarcomatous differentiation. Virchows Arch. 2006;448:85–9.

Feeley C, Gallagher P, Lang D. Osteosarcoma arising in a metastatic leiomyosarcoma. Histopathology. 2005;46:108–17.

Nicolas MM, Tamboli P, Gomez JA, Czerniak BA. Pleomorphic and dedifferentiated leiomyosarcoma: clinicopathologic and immunohistochemical study of 41 cases. Hum Pathol. 2010;41:663–71.

Oda Y, Miyajima K, Kawaguchi K, Tamiya S, Oshiro Y, Hachitanda Y, Oya M, Iwamoto Y, Tsuneyoshi M. Pleomorphic leiomyosarcoma: clinicopathologic and immunohistochemical study with special emphasis on its distinction from ordinary leiomyosarcoma and malignant fibrous histiocytoma. Am J Surg Pathol. 2001;25:1030–8.

Demicco EG, Boland GM, Brewer Savannah KJ, Lusby K, Young ED, Ingram D, Watson KL, Bailey M, Guo X, Hornick JL, van de Rijn M, Wang WL, Torres KE, Lev D, Lazar AJ. Progressive loss of myogenic differentiation in leiomyosarcoma has prognostic value. Histopathology. 2015;66:627–38.

Thông tin
Thông tin xuất bản

Clinical Sarcoma Research

Thông tin tác giả