BACKGROUND Histiocytic sarcoma is a rare malignant hematopoietic neoplasm with morphologic and immunohistochemical features of histiocytic differentiation, usually with unfavorable prognosis. Despite aggressive biological behavior, in subgroup of patients with localized disease, the prognosis can be very good. Few publications are available on localized cases of histiocytic sarcoma. These occur infrequently and continue to be a poorly-recognized morphological entity. CASE REPORT A 73-year old man treated for Parkinson syndrome presented with a tumor resistance on the dorsal surface of the left forearm. This lesion was clinically seen as an organized hematoma and was surgically resected. Histologically, the tumor was situated in the dermis and subcutis and it consisted of multiple neoplastic nodules. Vasoformative growth patterns with the vascular-like spaces containing erythrocytes and hemosiderin pigment presence simulated the morphology of angiosarcoma. Based on the immunohistochemical characteristics, we diagnosed the tumor as cutaneous histiocytic sarcoma. Genetic analysis revealed immunoglobulin heavy-chain gene rearrangement without any concomitant hematological malignancy. The patient demonstrated no systemic disease or impairment associated with diagnosed histiocytic sarcoma, and no recurrence has been found to date. CONCLUSIONS We report a case of primary cutaneous histiocytic sarcoma with an excellent outcome after surgical treatment only. Clinical data and histopathological and immunohistochemical evaluation were essential to rule out other malignant tumors in the differential diagnosis. Genetic analysis together with up-to-date knowledge and understanding of principles of tumorous transformations helped to diagnose this poorly-recognized entity with various clinical behaviors.

Department of Clinical and Molecular Pathology Medical School Palacky University Olomouc Czech Republic

Department of Clinical and Molecular Pathology Medical School University of Ostrava Ostrava Czech Republic

Department of Neurology Medical School University of Ostrava Ostrava Czech Republic

Department of Pathology CGB Laboratories a s Ostrava Vítkovice Czech Republic

Department of Pathology CGB laboratory a s Ostrava Vítkovice Czech Republic

See more in PubMed

Dalia S, Shao H, Sagatys E, et al. Dendritic cell and histiocytic neoplasms: Biology, diagnosis, and treatment. Cancer Control. 2014;21:290–300. PubMed

Dong A, Wang Y, Cui Y, et al. Enhanced CT and FDG PET/CT in histiocytic sarcoma of the pericardium. Clin Nucl Med. 2016;41:326–27. PubMed

Swerdlow H, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. 2017:468–70.

Andersen KF, Sjö LD, Kampmann P, et al. Histiocytic sarcoma: Challenging course, dismal outcome. Diagnostics (Basel) 2021;11:310. PubMed PMC

Feldman AL, Arber DA, Pittaluga S, et al. Clonally related follicular lymphomas and histiocytic/dendritic cell sarcomas: Evidence for transdifferentiation of the follicular lymphoma clone. Blood. 2008;111(12):5433–39. PubMed PMC

Alvaro T, Bosch R, Salvadó MT, et al. True histiocytic lymphoma of the stomach associated with low-grade B-cell mucosa-associated lymphoid tissue (MALT)-type lymphoma. Am J Surg Pathol. 1996;20(11):1406–11. PubMed

Feldman AL, Berthold F, Arceci RJ, et al. Clonal relationship between precursor T-lymphoblastic leukaemia/lymphoma and Langerhans-cell histiocytosis. Lancet Oncol. 2005;6(6):435–37. PubMed

Sohn BS, Kim T, Kim JE, et al. A case of histiocytic sarcoma presenting with primary bone marrow involvement. J Korean Med Sci. 2010;25:313–16. PubMed PMC

deMent SH. Association between mediastinal germ cell tumors and hematologic malignancies: An update. Hum Pathol. 1990;21:699–703. PubMed

Zafar A, Hanif F. Unusual presentation of a rare tumor: Histiocytic sarcoma presenting as a finger growth. Cureus. 2019;11(11):e6150. PubMed PMC

Wu W, Tanrivermis Sayit A, Vinters HV, et al. Primary central nervous system histiocytic sarcoma presenting as a postradiation sarcoma: Case report and literature review. Hum Pathol. 2013;44(6):1177–83. PubMed

Sato N, Arai E, Nakamura Y, et al. Primary cutaneous localized/clearly-outlined true histiocytic sarcoma: Two long-term follow-up cases. J Dermatol. 2020;47:651–53. PubMed

Jiang QM, Zhou WW, Song R, et al. [Histiocytic sarcoma: Clinicopathologic study of 4 cases.] Zhonghua Xue Ye Xue Za Zhi. 2012;33(9):751–55. [in Chinese] PubMed

Zheng YY, Zhou XG, Zhang SH, et al. [Histiocytic sarcoma: A clinicopatho-logical study of 6 cases.] Zhonghua Bing Li Xue Za Zhi. 2010 Feb;39(2):79–83. [in Chinese] PubMed

Trevisan F, Xavier CA, Pinto CA, et al. Case report of cutaneous histiocytic sarcoma: Diagnostic and therapeutic dilemmas. An Bras Dermatol. 2013;88(5):807–10. PubMed PMC

Hornick JL, Jaffe ES, Fletcher CD, et al. Extranodal histiocytic sarcoma: Clinicopathologic analysis of 14 cases of a rare epithelioid malignancy. Am J Surg Pathol. 2004;28(9):1133–44. PubMed

Ajabnoor R, Bell PD, Schiffman S, et al. Histiocytic sarcoma arising from a long bone: Report of two cases. Int J Surg Pathol. 2021;29:752–58. PubMed

Aytekin A, Ozet A, Bilgetekin I, et al. A metastatic histiocytic sarcoma case with primary involment of the tonsil. J Cancer Res Ther. 2020;16(3):665–67. PubMed

Saboo S, Krajewski KM, Shinagare AB, et al. Imaging features of primary extranodal histiocytic sarcoma: Report of two cases and a review of the literature. Cancer Imaging. 2012;12(1):253–58. PubMed PMC

Gergis U, Dax H, Ritchie E, et al. Autologous hematopoietic stem-cell transplantation in combination with thalidomide as treatment for histiocytic sarcoma: A case report and review of the literature. J Clin Oncol. 2011;29:e251–3. PubMed

Abidi MH, Tove I, Ibrahim RB, Maria D, Peres E. Thalidomide for the treatment of histiocytic sarcoma after hematopoietic stem cell transplant. Am J Hematol. 2007;82:932–33. PubMed

Idbaih A, Mokhtari K, Emile J-F, et al. Dramatic response of a BRAF V600E-mutated primary CNS histiocytic sarcoma to vemurafenib. Neurology. 2014;83:1478–80. PubMed

Gatalica Z, Bilalovic N, Palazzo JP, et al. Disseminated histiocytoses bio-markers beyond BRAFV600E: frequent expression of PD-L1. Oncotarget. 2015;6(23):19819–25. PubMed PMC

Tsirigotis P, Savani BN, Nagler A, et al. Programmed death-1 immune checkpoint blockade in the treatment of hematological malignancies. Ann Med. 2016;48:428–39. PubMed

Facchetti F, Pileri SA, Lorenzi L, et al. Histiocytic and dendritic cell neoplasms: What have we learned by studying 67 cases. Virchows Arch. 2017;471:467–89. PubMed