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Year : 2020  |  Volume : 7  |  Issue : 2  |  Page : 74-77

Histiocytic sarcoma: An encounter with a rarity

Department of Dermatology, Venereology and Leprosy, Government T. D. Medical College, Alappuzha, Kerala, India

Date of Submission09-Jan-2020
Date of Decision25-Jun-2020
Date of Acceptance31-Aug-2020
Date of Web Publication22-Dec-2020

Correspondence Address:
Dr. Neeraja S Kartha
Department of Dermatology, Venereology and Leprosy, Government T. D. Medical College, Alappuzha, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijdpdd.ijdpdd_4_20

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Histiocytic sarcoma is a rare hematolymphoid neoplasm originating from histiocytic cell clones. The lesions can occur in nodal or extranodal sites. A minority of cases present as unique cutaneous lesions. The definitive diagnosis is made by immunohistochemistry. The treatment depends on the severity of the disease. Herein, we report a case of histiocytic sarcoma in a 65-year-old female presenting with subcutaneous swellings and ulcerated coalescing nodules on the forearms and thighs with metastasis to the lung and treated with systemic chemotherapy.

Keywords: Chemoresistance, histiocytic sarcoma, immunohistochemistry

How to cite this article:
Kartha NS, Sobhanakumari K, Jose P. Histiocytic sarcoma: An encounter with a rarity. Indian J Dermatopathol Diagn Dermatol 2020;7:74-7

How to cite this URL:
Kartha NS, Sobhanakumari K, Jose P. Histiocytic sarcoma: An encounter with a rarity. Indian J Dermatopathol Diagn Dermatol [serial online] 2020 [cited 2021 Sep 27];7:74-7. Available from: https://www.ijdpdd.com/text.asp?2020/7/2/74/304336

  Introduction Top

Histiocytic sarcoma is a non-Langerhans cell histiocytic disorder of unknown cause. Although it can occur in nodal and extranodal sites, the latter is frequent and the clinical course is generally aggressive. Persistent clinical and histopathological disparities, along with overall rarity, continue to be a major hurdle for the diagnosis of this neoplasm. Till date, only a few hundred cases have been reported worldwide.[1] Exclusive cutaneous involvement is very rare, with skin involvement seen only in 7% of the cases.[2] We report a case of histiocytic sarcoma affecting the skin.

  Case Report Top

A 65-year-old female with no comorbidities presented to our department with swellings over the right forearm and right thigh of 8-month duration. There was a rapid increase in size with mild pain of 5-month duration and recent loss of appetite and weight of 3-month duration. On general examination, the patient was poorly nourished with pallor and multiple significantly enlarged axillary lymphnodes on the right side. Dermatological examination revealed two well-defined, fixed, and slightly tender swellings of sizes 14 cm × 7 cm over the posterior aspect of the right upper thigh and 10 cm × 3 cm on the right forearm. All were hard in consistency with small ulcerations on the surface [Figure 1] and [Figure 2]. Systemic examination was normal. Differential diagnosis of mycosis fungoides, B-cell lymphoma, leukemia cutis, and cutaneous metastasis was made, and the patient was investigated. Routine hemogram was normal except for a low hemoglobin level (8 g/dl). Ultrasonography abdomen was normal. Fine-needle aspiration cytology (FNAC) from the forearm swelling showed soft-tissue neoplasm, possibly histiocytic origin. An incision biopsy was taken from the swelling and sent for histopathology and immunohistochemistry (IHC).
Figure 1: Hard swellings with surface showing small ulcerations over the posterior aspect of the right upper thigh

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Figure 2: Hard swellings with surface showing small ulcerations over the right forearm

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Histopathology revealed diffuse and nodular dermal and subcutaneous histiocytic infiltrates composed of pleomorphic spindle-shaped cells with large vesicular nuclei, prominent nucleoli, and eosinophilic foamy cytoplasm with occasional multinucleated giant cells suggesting a histiocytic origin [Figure 3], [Figure 4], [Figure 5].
Figure 3: Photomicrograph of the swelling showing diffuse infiltrates of highly pleomorphic and spindle cells with frequent mitoses noted (H and E, ×10)

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Figure 4: Photomicrograph of the swelling showing dermal and subcutaneous histiocytic infiltrates (H and E, ×40)

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Figure 5: Photomicrograph of the swelling showing foamy macrophages, spindle-shaped cells, and multinucleated giant cells (H and E, ×100)

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IHC showed positivity for CD68 and vimentin, further strengthening the possibility of a histiocytic origin [Figure 6]a and [Figure 6]b. S100 and smooth muscle actin were negative, thereby ruling out Langerhans cell and smooth muscle origin, respectively [Figure 6]c and [Figure 6]d.
Figure 6: Immunohistochemistry showing cells. (a) Positive for CD68. (b) Positive for Vimentin. (c) Negative for S100. (d) Negative for smooth muscle actin

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Magnetic resonance imaging showed extension of swelling into the subcutaneous plane with infiltration into the intermuscular plane and right axillary lymph node, favoring the possibility of a malignant soft-tissue tumor [Figure 7] and [Figure 8].
Figure 7: Magnetic resonance imaging showing infiltration of the forearm swelling into the intermuscular plane

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Figure 8: Magnetic resonance imaging showing infiltration into the right axillary lymph node

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Hence, a final diagnosis of histiocytic sarcoma was made. Metastatic workup revealed a nodule in the lower zone of the right lung which was confirmed by computerized tomography thorax [Figure 9] and [Figure 10].
Figure 9: Chest X Ray showing metastatic nodule in the right lower lobe

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Figure 10: Computed tomography thorax showing metastatic nodule in the right lower lobe

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The patient was started on systemic chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisolone [CHOP] regimen) and is currently on her 16th cycle. On follow-up, the swellings are remaining static, suggesting the possibility of chemoresistance of the tumor.

  Discussion Top

Histiocytic sarcoma is a malignant neoplasm showing morphologic and immunophenotypic features of mature tissue histiocytes.[3] The term “histiocytic sarcoma” was introduced in 1970 by Mathé et al.[4] It is usually seen in adults in the fifth decade, with a slight male preponderance. Etiology of this disorder is unknown. Around 25% of the cases transdifferentiate from hematolymphoid malignancies, especially those of B-cell origin including follicular lymphoma, diffuse large B-cell lymphoma, marginal B zone lymphoma, etc. Clonal immunoglobulin and T-cell receptor gene rearrangements are usually seen in such transdifferentiated cases and, at times, in some sporadic cases.[5] Neoplastic histiocytes retain some genetic alterations of the prior B-cell lymphoma including t(14; 18) of follicular lymphoma, del(13q) of chronic lymphocytic leukemia, BRAFV600E mutation in 63% cases, and in rare cases, an isochromosome 12p which is identical to that seen in germ cell tumors.[6] The majority of the cases present in extranodal sites, with gastrointestinal tract, skin, and soft-tissues being the most frequently affected. Others present with lymphadenopathy.[5] Cutaneous involvement occurs alone which is rare (primary cutaneous histiocytic sarcoma) or as a part of systemic involvement.[7] It can be seen as subcutaneous nodules with ulceration or rarely as a maculopapular rash.[8] IHC shows positivity for CD68, CD163, lysozyme, CD45, HLA-DR, CD14, CD15, CD11c, and α1-antitrypsin with negative staining for CD1a, S100, B-cell, and T-cell markers.[5],[6] The chance of survival in diagnosed cases is bleak as 60% of the cases present with metastasis at the time of presentation and the lack of a standardized treatment regimen further contributes. The preferred treatment is wide surgical excision with/without chemotherapy or radiotherapy. Chemotherapy is usually non-Hodgkin's lymphoma protocol, which is the CHOP regimen or ifosfamide, carboplatin, and etoposide regimen.[9]

Our patient was an elderly female presenting with subcutaneous swellings on the skin with surface ulcerations along with involvement of regional lymph nodes and lungs. FNAC and histopathology strengthened the possibility of histiocytic origin which was confirmed by IHC. The presence of metastasis and chemoresistance further contributed to the poor prognosis in our patient.

Histiocytic sarcoma is an underreported entity in dermatology due to paucity of cases with cutaneous involvement and frequent misdiagnosis. There are only a few reported cases in Indian literature till date. This clinical vignette has definitely brought out the indispensable role of IHC in the diagnosis of extremely rare entities such as histiocytic sarcoma, where our clinical acumen is tested to the hilt. In spite of a grave prognosis, early detection of primary cutaneous disease has a favorable outcome if complete surgical excision is performed, thereby having a significant contribution in reducing mortality.[10]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Vos JA, Abbondanzo SL, Barekman CL, Andriko JW, Miettinen M, Aguilera NS. Histiocytic sarcoma: A study of five cases including the histiocyte marker CD163. Mod Pathol 2005;18:693-704.  Back to cited text no. 1
Trevisan F, Xavier CA, Pinto CA, Cattete FG, Stock FS, Martins ML. Case report of cutaneous histiocytic sarcoma: Diagnostic and therapeutic dilemmas. An Bras Dermatol 2013;88:807-10.  Back to cited text no. 2
Schnettler K, Salomone C, Valbuena JR. Cutaneous histiocytic sarcoma: Report of one case. Rev Med Chil 2009;137:547-51.  Back to cited text no. 3
Mathé G, Gerard-Marchant R, Texier JL, Schlumberger JR, Berumen L, Paintrand M. The two varieties of lymphoid tissue “reticulosarcomas”, histiocytic and histioblastic types. Br J Cancer 1970;24:687-95.  Back to cited text no. 4
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Geneva: IARC WHO Classification of Tumours; 2008. p. 439.  Back to cited text no. 5
Pan Z, Xu M. Histiocytic and Dendritic cell neoplasms. Surg Pathol Clin. 2019;12(3):805-29.  Back to cited text no. 6
Arai E, Yamamoto T, Kayano H. Primary cutaneous true histiocytic sarcoma: A case with an indolent course. J Clin Exp Hematopathol 2003;43:35-40.  Back to cited text no. 7
Osborne BM, Mackay B. True histiocytic lymphoma with multiple skin nodules. Ultrastruct Pathol 1994;18:241-6.  Back to cited text no. 8
Alexander M, Daniels JR. Chemotherapy of malignant histiocytosis in adults. Cancer 1977;39:1011-7.  Back to cited text no. 9
Ducatman BS, Wick MR, Morgan TW, Banks PM, Pierre RV. Malignant histiocytosis: A clinical, histologic, and immunohistochemical study of 20 cases. Hum Pathol 1984;15:368-77.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]


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