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CASE REPORT
Year : 2020  |  Volume : 16  |  Issue : 3  |  Page : 665-667

A metastatic histiocytic sarcoma case with primary involvement of the tonsil


1 Department of Internal Medicine, Division of Medical Oncology, Faculty of Medicine, Gazi University, Ankara, Turkey
2 Department of Pathology, Faculty of Medicine, Gazi University, Ankara, Turkey

Date of Submission09-May-2016
Date of Decision13-Jun-2016
Date of Acceptance30-Jun-2016
Date of Web Publication16-Aug-2016

Correspondence Address:
Aydin Aytekin
Department of Internal Medicine, Division of Medical Oncology, Faculty of Medicine, Gazi University, Ankara
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.188435

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 > Abstract 


Histiocytic sarcoma (HS) is an extremely rare and aggressive hematopoietic tumor. Although it can be seen at any anatomic location, the most common primary sites are skin as extranodal region, locations including the lymph nodes and gastrointestinal tract. To the best of our knowledge, in light of PubMed search, this is the first primary tonsillar HS case presented with disseminated metastases at the time of diagnosis. A 58-year-old male patient applied with swelling on the right side of the neck, difficulty in swallowing, and weight loss. Positron emission tomography computed tomography was performed and increased pathological 18F fluorodeoxy D glucose uptake was detected in the right palatine tonsil, bilateral cervical multiple lymph nodes, liver masses, intra abdominal lymph nodes, and nodular lesion in the left adrenal gland. Tonsillectomy was performed and the pathological result was reported as HS. The patient did not respond to any treatment and had died after 5 months from the date of diagnosis. In conclusion, HS is generally diagnosed at advanced stage, it has limited chemotherapy response and high mortality rates. To understand this rare disease's pathophysiological and clinical features, further investigations are needed.

Keywords: Histiocytes, histiocytic sarcoma, metastatic, tonsil


How to cite this article:
Aytekin A, Ozet A, Bilgetekin I, Ogut B, Ciltas A, Benekli M. A metastatic histiocytic sarcoma case with primary involvement of the tonsil. J Can Res Ther 2020;16:665-7

How to cite this URL:
Aytekin A, Ozet A, Bilgetekin I, Ogut B, Ciltas A, Benekli M. A metastatic histiocytic sarcoma case with primary involvement of the tonsil. J Can Res Ther [serial online] 2020 [cited 2020 Aug 9];16:665-7. Available from: http://www.cancerjournal.net/text.asp?2020/16/3/665/188435




 > Introduction Top


Histiocytic sarcoma (HS) is an extremely rare and aggressive hematopoietic tumor, with morphologic and immunophenotypic characteristics of mature tissue histiocytes.

There is an inconsistency between terminology and diagnostic criteria of lesions. To date, HS has variously been defined as histiocytic lymphoma, malignant histiocytosis, histiocytic medullary reticulosis, reticulum sarcoma, and regressing atypical histiocytosis.[1]

According to the World Health Organization classification, HS is characterized as a malignant proliferation of cells that has similar morphology and immunophenotyping with mature histiocytes.[2] Once, HS was known as true histiocytic lymphoma. It seems to be an uncommon malignancy that accounts for <1% of hematologic malignancies. Although adult male patients are mostly affected, it could not be limited to a specific age group.[3],[4] The initial symptoms may be fever, night sweats, palpable lymphadenopathy (LAP), or localized pain.[3],[4],[5] Although it can be seen at any anatomic location, the most common primary sites are skin as extranodal region, locations including the lymph nodes and gastrointestinal tract. It has been reported that also central nervous system, spleen, bone marrow, and thyroid are involved. Disease may be local or disseminated.[3],[5],[6],[7]

Diagnosis requires the exclusion of other diseases with a wide immunophenotypic study. We want to report the first case according to PubMed search that presented with metastasis at the time of diagnosis and which is also the second case with primary involvement of the tonsil.


 > Case Report Top


A 58-year-old male patient who had no immunosuppressive or immune defective disease referred with swelling on the right side of the neck, difficulty in swallowing, and weight loss. In magnetic resonance imaging,

22 mm × 14 mm × 12 mm mass lesion was observed in the right tonsil [Figure 1]a and bilateral multiple LAPs largest of which was the size of 4 cm × 2 cm in the right cervical level 2–3 [Figure 1]b were revealed. Positron emission tomography-computed tomography was performed and high pathologic 18F fluorodeoxy D glucose uptake was detected in the right palatine tonsils (maximum standardized uptake value [SUVmax]: 10.2), bilateral multiple lymph nodes in the neck (SUVmax: 12.2), metastatic lesions in liver (SUVmax: 6.9); in intra-abdominal, celiac, aortocaval, and the para-aortic lymph nodes (SUVmax: 6.6); a nodular lesion in the left adrenal gland (SUVmax: 5.8) [Figure 2]a and [Figure 2]b. Tonsillectomy was performed and the pathological result was reported as HS which is vimentin, CD68, CD163, fascin, lysozyme, and CD4 positive by immunohistochemistry; the tumoral infiltration whose Ki-67 proliferation index was 70% was considered as HS after other causes were excluded [Figure 3]a, [Figure 3]b, [Figure 3]c. The patient underwent cyclophosphamide, doxorubicin, vincristine, prednisone chemotherapy. After two cycles, progression was identified in examination and ifosfamide, carboplatin, etoposide regimen was considered to be administered. Nevertheless, chemotherapy could not be administered to the patient because of hyperbilirubinemia and elevated liver function tests. The patient was treated with radiotherapy to his great masses of the liver for palliative care due to the progression of hepatic failure. After undergoing palliative care, our patient, who had about 5-month survival from the date of diagnosis, had died shortly.
Figure 1: (a) Magnetic resonance imaging showing right tonsillar lesion. (b) Magnetic resonance imaging showing right cervical lymphadenopathy

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Figure 2: (a) Positron emission tomography maximum intensity projection imaging showing primary tonsillar lesion and cervical lymphadenopathy. (b) Whole body positron emission tomography fusion imaging showing primary and metastatic lesions

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Figure 3: (a) Pleomorphic tumor cells with eosinophilic cytoplasm and large nuclei involving prominent nucleoli (H and E, ×400). (b) (×200) Diffuse cytoplasmic positivity of CD68. (c) (×200) diffuse cytoplasmic positivity of CD163

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 > Discussion and Conclusion Top


Although tumors are seen in a wide range of ages (6 months–89 years, median age 46), due to a small peak between 0 and 29 years and a larger peak between 50 and 69 years of age, it is said to have bimodal age distribution. Some cases, such as B- or T-cell lymphoma/leukemia or follicular lymphoma, chronic lymphocytic leukemia, mantle cell lymphoma, mucosal associated extranodal marginal zone lymphoma, splenic marginal zone lymphoma, and diffuse large B mature as cell lymphoma, can occur simultaneously with mature B-cell lymphoma or subsequently.[1]

HS is diagnosed using an extensive immunohistochemical panel with the recognition of atypical histiocytic morphology of tumor cells and showing the expression markers associated with histiocytes after excluding the other causes. Differential diagnosis should include reactive histiocytic proliferation, dendritic cell neoplasm, especially large cell non-Hodgkin's lymphoma such as diffuse large B-cell lymphoma and anaplastic large cell lymphoma, malignant melanoma, undifferentiated large cell carcinoma, and monocytic leukemia.[1]

Due to the development of immunohistochemical techniques, the identification of tumor and its primary tissue has been facilitated. So far, the International Lymphoma Study Group emphasized immunophenotypic markers (e.g., CD68, CD1a, S-100, CD21, CD35) to determine the dendritic cell sarcoma and the HS correctly. More recently, Vos et al. showed that hemoglobin scavenger receptor (CD163) is a specific marker that identifies cells with histiocytic origin. As a result, it can be said that CD163 and CD68 are the best diagnostic markers in the diagnosis of HS.[8]

HS generally presents at advanced stage, response to chemotherapy is limited, and mortality rate is high. Most patients die within 2 years due to progressive disease. Nevertheless, some patients may have a moderate course of disease with response to chemotherapy and/or radiotherapy.[3] In addition to this, better disease-free survival has been reported in the subgroup presenting with localized disease.[3],[8] Therefore, the stage at the time of diagnosis seems to be effective in prognosis. However, there is no consensus on the prognostic factors and standard therapeutic approaches because of the rarity of the disease. For this reason, lymphoma-based chemotherapies have been attempted to be used. Good results have been reported with thalidomide treatment.[5]

In our case, the patient was admitted with local symptoms such as dysphagia and systemic symptoms such as weight loss. Tonsillar mass, regional LAP, and distant metastasis was detected. To the best of our knowledge, this is the first primary HS case in the literature presenting with disseminated metastases at the time of diagnosis. The previously reported case was partly localized disease with a complete response to treatment.[9]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 > References Top

1.
Takahashi E, Nakamura S. Histiocytic sarcoma: An updated literature review based on the 2008 WHO classification. J Clin Exp Hematop 2013;53:1-8.  Back to cited text no. 1
    
2.
Vardiman JW. The World Health Organization (WHO) classification of tumors of the hematopoietic and lymphoid tissues: An overview with emphasis on the myeloid neoplasms. Chem Biol Interact 2010;184:16-20.  Back to cited text no. 2
    
3.
Hornick JL, Jaffe ES, Fletcher CD. Extranodal histiocytic sarcoma: Clinicopathologic analysis of 14 cases of a rare epithelioid malignancy. Am J Surg Pathol 2004;28:1133-44.  Back to cited text no. 3
    
4.
Hayase E, Kurosawa M, Yonezumi M, Suzuki S, Suzuki H. Aggressive sporadic histiocytic sarcoma with immunoglobulin heavy chain gene rearrangement and t (14;18). Int J Hematol 2010;92:659-63.  Back to cited text no. 4
    
5.
Gergis U, Dax H, Ritchie E, Marcus R, Wissa U, Orazi A. 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.  Back to cited text no. 5
    
6.
Sohn BS, Kim T, Kim JE, Bae E, Park CJ, Huh J, et al. Acase of histiocytic sarcoma presenting with primary bone marrow involvement. J Korean Med Sci 2010;25:313-6.  Back to cited text no. 6
    
7.
Zhang D. Histiocytic sarcoma arising from lymphomas via transdifferentiation pathway during clonal evolution. Leuk Lymphoma 2010;51:739-40.  Back to cited text no. 7
    
8.
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. 8
    
9.
Chen X, Zhang L, Wang J, Gu Y, Tuan J, Ma X, et al. Complete response after chemotherapy and radiotherapy of a tonsillar histiocytic sarcoma with regional lymph node involvement: A case report and review of the literature. Int J Clin Exp Med 2015;8:16808-12.  Back to cited text no. 9
    


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