16 April 2022: Articles
Histiocytic Sarcoma with an Unusual Clinical Manifestation Imitating Malignant Vascular Tumor: A Case Report
Unknown etiology, Unusual clinical course, Challenging differential diagnosis, Rare disease
Róbert Ondruššek12CE*, Jana Žmolíková3D, Jarmila Šimová3E, Petra Bartová45B, Pavel Hurník34B, Magdalena Uvírová34B, Dušan Žiak34EDOI: 10.12659/AJCR.935824
Am J Case Rep 2022; 23:e935824
Abstract
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.
Keywords: Diagnosis, Histiocytic Sarcoma, Histology, Diagnosis, Differential, Humans, Male, Skin Neoplasms, Vascular Neoplasms
Background
Histiocytic sarcoma (HS) is a rare malignant tumor accounting for less than 1% of all hematological neoplasms [1]. The typical histiocytic differentiation is usually found at extranodal sites, including skin, gastrointestinal tract, soft tissues, and other organs, and, very rarely, in the pericardium [2]. These tumors can occur in wide age range, with an average age of 52 years, with a male predilection according to some studies [3]. The etiology and pathogenesis are unknown. The literature reports presumed transdifferentiation of a preexisting hematolymphoid disorder in approximately 25% of the cases [4–6]. Localized disease of skin and connective soft tissue has a better prognosis with a longer overall survival in patients receiving only surgical therapy. Disseminated disease with involvement of hematopoietic organs usually has poor outcome, despite available chemotherapy. The present case of localized cutaneous histiocytic sarcoma had an excellent prognosis. Also, we would like to demonstrate the diagnostic challenge undertaken here to specify the diagnosis and to distinguish from other vascular malignancies known to have more aggressive behavior.
Case Report
A 73-year-old man had been treated for Parkinson syndrome with antiparkinsonian therapy (L-DOPA) since 1999. He received surgical deep brain stimulation for impaired body motion and late complications of movement disorder as part of neurological treatment in 2008. He had been treated for psoriasis vulgaris and had nasal barriers for repair of a deviated nasal septum. He was trained as an electrician in his younger years and later he also worked as a gardener and inspection technician. No allergies were reported. His family history revealed his father had neurological impairment of fine movement-shaking disturbances and he died of non-specified gastric complications. Mother was treated for bowel cancer and died of complications related to a fractured femoral neck. The patient’s current disease started when he reported a lump on the dorsal surface of the left forearm. He has noticed this skin resistance first 9 months before going to the doctor’s office. The tumor was 5 cm in diameter and was clinically assessed as an organized hematoma. The lesion was surgically resected in December 2019. There were no need for re-excision, as the surgical margins were tumor-free when we performed histopathological examination of this specimen. Macroscopically, the tumor appeared to be fairly localized, of firm consistency, and discolored yellow-to-grey. Microscopically, the tumor in the dermis and subcutis consisted of multiple neoplastic nodules separated with fibrous septa. There were also reticular and vasoformative growth patterns, numerous areas with vascular-like spaces filled with erythrocytes (Figure 1A). Within solid parts, cord-like changes were present and there were deposits of hemosiderin-laden macrophages (siderophages) in fibrous neoplastic stroma within the tumor (Figure 1B). The neoplastic cells were monomorphic with occasional round-to-oval larger cells with abundant eosinophilic cytoplasm (Figure 1C, marked by blue arrows) and with osteoclast-like cells (Figure 1C, marked by yellow arrows). Some of neoplastic nuclei showed irregular folds. Occasional hemophagocytosis was spotted (Figure 1D). Adjacent mild inflammatory infiltrate consisting of lymphocytes was seen. The featured hemosiderin pigment and the above-mentioned growth patterns simulated the morphology of angiosarcoma. Based on the extensive immunohistochemical evaluation, we made a diagnosis of histiocytic sarcoma. Histiocytic markers CD68 a CD163, LCA, CD45RO, CD4, and lysozyme were expressed by the tumor. All other markers – CK AE1/3, CK5/6, CD3, CD20, CD23, CD1a, CD34, ERG, factor VIII, CD30, CD117, CD99, S100, PAX5, SOX10, smooth muscle actin, EMA, caldesmon H, desmin, thyroglobulin and melanocyte markers – were negative. Proliferative activity in Ki67 (mitotic index) was around 15%. Histopathological assessment was complimented by genetic testing. FISH (fluorescence in situ hybridization) demonstrated an immunoglobulin heavy-chain (
Discussion
By definition, HS is a malignant proliferation of cells demonstrating pathological and immunohistochemical signs of mature tissue histiocytes with expression of 1 or more histiocytic markers, including CD68, lysozyme, and CD163, with absence of Langerhans cells, follicular dendritic cells, and myeloid cell markers [3]. HS usually lacks clonal
Conclusions
Localized extranodal cutaneous histiocytic sarcoma is an uncommon and unique malignancy with specific histopathological features and genetic characteristics. It is important to distinguish this rare entity from conditions mimicking angiosarcoma and malignant vascular tumors. The surgical resection provided clear specimen margins confirmed by histology, which has prognostic importance and makes surgery a possible curable approach, particularly in patients with localized HS. This strongly suggests the existence of a group of curable patients with excellent prognosis
Figures
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