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Year : 2020  |  Volume : 21  |  Issue : 1  |  Page : 59-62

A Rare Entity of S100(−)/cluster of Differentiation 68(+) Jaw Myxoid-Cellular Neurothekeoma: A Case Report and Literature Review

1 Department of Pediatric Hematology-Oncology, University Malaya Medical Center, Kuala Lumpur, Malaysia
2 Department of Pathology, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia

Date of Submission25-Aug-2019
Date of Decision20-Sep-2019
Date of Acceptance27-Nov-2019
Date of Web Publication24-Dec-2019

Correspondence Address:
Dr Vida Jawin
Department of Pediatric Hematology-Oncology, Level 9, KWKK Block, University Malaya Medical Center, Kuala Lumpur
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_87_19

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Neurothekeomas are rare, benign soft tissue tumors with fairly distinctive histomorphological features. They arise from the nerve sheath (nerve sheath myxoma/myxoid type) or fibrohistiocytic matrix (cellular type) and commonly involve the face, neck, and upper extremities in young adults. We report a rare, mixed-type neurothekeoma (showing both myxoid and cellular features), affecting the jaw of an 11-year-old girl. Histology was consistent with typical myxoid pattern, while immunohistochemistry suggested a fibrohistiocytic origin (cellular pattern). We excised the mass completely without any evidence of recurrence after 6 months.

Keywords: Fibrohistiocytic, jaw, mixed, myxoid, neurothekeoma

How to cite this article:
Jawin V, Chun FJ, Hooi TK, Rajagopal R. A Rare Entity of S100(−)/cluster of Differentiation 68(+) Jaw Myxoid-Cellular Neurothekeoma: A Case Report and Literature Review. Indian J Paediatr Dermatol 2020;21:59-62

How to cite this URL:
Jawin V, Chun FJ, Hooi TK, Rajagopal R. A Rare Entity of S100(−)/cluster of Differentiation 68(+) Jaw Myxoid-Cellular Neurothekeoma: A Case Report and Literature Review. Indian J Paediatr Dermatol [serial online] 2020 [cited 2020 Apr 1];21:59-62. Available from: http://www.ijpd.in/text.asp?2020/21/1/59/273843

  Introduction Top

Neurothekeomas are rare, benign soft tissue tumors commonly affecting the adolescents, usually affecting the head, neck, and upper extremities.[1] Histologic variants include myxoid, cellular, and myxoid-cellular (mixed) type.[1],[2] To the best of our knowledge, there are only five reports of mixed variety (myxoid-cellular) of this tumor in the English literature to date [Table 1].[3],[4],[5],[6],[7] Among them, only one case has been reported in the pediatric age group.[6] Thus, we report the rare myxoid-cellular/mixed variety of this uncommon tumor affecting the jaw of an 11-year-old female and discuss the histological and immunohistochemical findings along with the review of literature.
Table 1: Similar case reports[3],[4],[5],[6],[7]

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  Case Report Top

An 11-year-old girl presented with an asymptomatic, progressively enlarging mass affecting the superolateral aspect of her jaw for the last 6 years. There was no history of constitutional symptoms or trauma. Physical examination revealed a palpable pinkish-purple, nontender nodule over her right jaw [Figure 1]. The nodule was firm, mobile, nonfluctuant about 1 cm × 1 cm in dimension, without any overlying skin inflammation. Residual systemic examination was unremarkable. Routine biochemistry including full blood count and lactate dehydrogenase was within normal limits. We performed complete excision with biopsy and advised follow-up after 6 months. Histopathology demonstrated a well-circumscribed multilobulated dermal tumor separated by fibrous septae [Figure 2]a, exhibiting spindle-to-stellate–shaped cells with scanty fibrocollagenous tissue and moderate amount of fibrillary eosinophilic cytoplasm embedded in abundant myxoid stroma [Figure 2]b and [Figure 2]c. Resection margin was clear from tumor. Immunohistochemical staining was positive for cluster of differentiation (CD) 68 and negative for S100 and glial fibrillary acidic protein (GFAP) [Figure 3]a and [Figure 3]b. In addition, cytokeratin and smooth muscle actin staining were negative, excluding tumor of neural, epithelial, or muscle origin. Ki-67 proliferative index was <5%. Although histopathology showed typical features of myxoid variant, we confirmed the diagnosis of myxoid-cellular (mixed-type) neurothekeoma based on positive staining for fibrohistiocytic marker (CD68). No evidence of recurrence was noted 6 months postsurgery.
Figure 1: A solitary, 1 cm × 1 cm nodule over the right jaw

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Figure 2: (a) The tumor is in dermis with mildly cellular areas separated by fibrous septae giving rise to a well-circumscribed multilobulated mass architecture (H and E, ×20), (b and c) stellate and spindle-shaped cells with mild nuclear pleomorphism, embedded within abundant myxoid stroma of > 50% myxoid matrix and moderate amount of eosinophilic cytoplasm (H and E, b, ×40, c, ×100)

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Figure 3: (a) The tumor cells showed the presence of cluster of differentiation 68-positive cells (×100), (b) the tumor cells were negative for S100 protein (×100)

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  Discussion Top

Neurothekeomas are rare, benign soft tissue tumors with unknown histogenesis.[8] Although historically linked with nerve sheath myxoma and often considered synonymous, recent immunohistochemistry studies have separated these two and established fibrohistiocytic differentiation to be its hallmark characteristic.[2],[3],[9] Clinical features include a flesh-colored or slightly erythematous slow-growing papule or nodule of soft consistency.[9]

Neurothekeomas have been subclassified histopathologically into three groups, depending on the amount of myxoid matrix-myxoid type (>50% myxoid matrix), mixed type (>10% but ≤50% myxoid matrix), and cellular type (≤10% myxoid matrix).[2] The typical histological features included a lobulated, well-circumscribed proliferation of stellate, spindled, or epithelioid cells arranged in the nests and cords with a mucinous, myxoid background containing dermal collagen bundles.[10]

The myxoid variant shows greater degree of myxomatous changes (>50%), less cellularity with well-circumscribed spindle-to-stellate–shaped cells in myxoid matrix and multinucleated giant cells.[2],[11],[12] The tumor cells are positive for nerve-origin cell markers S100, GFAP, nerve growth factor receptor, collagen type IV, and CD57.[2],[8],[13] However, they are negative for epithelial membrane antigen or markers of histiocytic differentiation.[4],[5],[11] Interestingly, several authors have reported “S100-negative” myxoid neurothekeoma [4],[5] [Table 1].

In contrast, the cellular types are nonencapsulated, poorly circumscribed, and typically composed of fascicles of spindle-shaped to epithelioid tumor cells with eosinophilic cytoplasm and sparse to absent mucinous matrix.[12],[14] The tumor cells are not stained with nerve-origin cell markers but show reactivity with fibrohistiocytic differentiation markers, NK1-C3 (CD63), CD10, and CD68, and the pan-monocyte marker, Ki-M1p.[11],[14],[15] The diagnosis of cellular neurothekeoma requires immunohistochemistry to identify the fibrohistiocytic markers in addition to histologic appearance.[16],[17] The mixed-type neurothekeoma usually shows overlapping features of both variants with interspersed cellular and myxoid regions.[12] Immunohistochemistry staining is often confusing, revealing irregular or absent reactivity to S100 protein, smooth muscle actin, and positive reaction for fibrohistiocytic differentiation markers.[12]

Strumìa et al. and Henmi et al. reported two cases of S100-negative myxoid neurothekeoma despite distinct myxoid-type by histology.[4],[5] However, fibrohistiocytic marker such as CD68 was not performed in these two cases.[4],[5] Furthermore, another three case reports with contradictory morphological and immunohistochemical findings have been reported (typical myxoid-type histologic findings but negative for S100 protein and positive for fibrohistiocytic staining [CD68, NK1/C3, and Ki-M1P]), similar to our case [Table 1].[3],[6],[7]

In our case, histopathology showed spindled-to-stellate cells embedded in abundant myxoid stroma (>50% myxoid matrix), consistent with myxoid variant [Figure 2]b and [Figure 2]c. However, immunohistochemical staining was suggestive of cellular variant (S100-negative; CD68-positive) [Figure 3]a and [Figure 3]b. Thus, a diagnosis of myxoid-cellular (mixed type) neurothekeoma was made. Complete excision was successful without any recurrence after 6 months.

Complete excision is the treatment of choice, irrespective of its type.[18] There is no clear consensus on extent of resection margin, but clear microscopic margin is adequate.[18] Few studies have evaluated its prognosis to record a recurrence rate of around 3%, especially in the presence of risk factors such as myxoid type, female gender, facial location, younger age at diagnosis, positive margins, and absence of fat tissue in the sample.[2],[9],[18]

  Conclusion Top

Pediatric myxoid-cellular/mixed neurothekeoma is rare. Myxoid-type neurothekeoma demonstrates abundant myxoid matrix histologically, while fibrohistiocytic differentiation markers are necessary to diagnose the cellular variant. In the absence of S100 myxoid neurothekeoma, it is essential to perform immunohistochemical staining with fibrohistiocytic markers to differentiate mixed-type neurothekeoma for proper evaluation of recurrence risk.

This case has been highlighted to report a rare variant of an uncommon tumor in the pediatric age group.

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.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Seo BF, Kang HW, Lee JY, Kwon H, Jung SN. Ankle neurothekeoma: A case report. J Foot Ankle Surg 2013;52:678-80.  Back to cited text no. 1
Fetsch JF, Laskin WB, Hallman JR, Lupton GP, Miettinen M. Neurothekeoma: An analysis of 178 tumors with detailed immunohistochemical data and long-term patient follow-up information. Am J Surg Pathol 2007;31:1103-14.  Back to cited text no. 2
Yun SJ, Park HS, Lee JB, Kim SJ, Lee SC, Won YH. Myxoid cellular neurothekeoma: A new entity of S100-negative, CD68-positive myxoid neurothekeoma. Ann Dermatol 2014;26:510-3.  Back to cited text no. 3
Strumìa R, Lombardi AR, Cavazzini L. S-100 negative myxoid neurothekeoma. Am J Dermatopathol 2001;23:82-3.  Back to cited text no. 4
Henmi A, Sato H, Wataya T, Inaniwa Y, Mori Y. Neurothekeoma. Report of a case with immunohistochemical and ultrastructural studies. Acta Pathol Jpn 1986;36:1911-9.  Back to cited text no. 5
Han TY, Han B, Lee YC, Lee JH, Son SJ. A Rare Case of Atypical Myxoid Cellular Neurothekeoma in a 6-Year-Old Girl. Pediatr Dermatol 2016;33:e123-4.  Back to cited text no. 6
Tas B, Altinay S, Azakli HN. A painful S100(-)/CD68(+) Myxoid- Hypocellularneurothekeoma: A case review through an unusual nerve sheath tumor. West Indian Med J 2015. DOI: 10.7727/wimj.2015.605.  Back to cited text no. 7
Papadopoulos EJ, Cohen PR, Hebert AA. Neurothekeoma: Report of a case in an infant and review of the literature. J Am Acad Dermatol 2004;50:129-34.  Back to cited text no. 8
Bhat A, Narasimha A, Vijaya C, Sundeep VK. Nerve sheath myxoma: Report of a rare case. J Clin Diagn Res 2015;9:ED07-9.  Back to cited text no. 9
Gallager RL, Helwig EB. Neurothekeoma-a benign cutaneous tumor of neural origin. Am J Clin Pathol 1980;74:759-64.  Back to cited text no. 10
Rudolph P, Schubert C. Myxoid cellular neurothekeoma. Am J Dermatopathol 2002;24:92-3.  Back to cited text no. 11
Müller CS, Tilgen W, Kutzner H, Pföhler C. Recurring mixed-type neurothekeoma of the face. Dermatoendocrinol 2009;1:220-2.  Back to cited text no. 12
Oh SH, Lee HJ, Chang SE, Lee MW, Choi JH, Moon KC, et al. A case of cellular neurothekeoma. Korean J Dermatol 2006;44:1126-9.  Back to cited text no. 13
Emami N, Zawawi F, Ywakim R, Nahal A, Daniel SJ. Oral cellular neurothekeoma. Case Rep Otolaryngol 2013;2013. doi: 10.1155/2013/935435.  Back to cited text no. 14
Busam KJ, Mentzel T, Colpaert C, Barnhill RL, Fletcher CD. Atypical or worrisome features in cellular neurothekeoma: A study of 10 cases. Am J Surg Pathol 1998;22:1067-72.  Back to cited text no. 15
Mahalingam M, Alter JN, Bhawan J. Multiple cellular neurothekeomas-a case report and review on the role of immunohistochemistry as a histologic adjunct. J Cutan Pathol 2006;33:51-6.  Back to cited text no. 16
Chang SE, Lee TJ, Ro JY, Choi JH, Sung KJ, Moon KC, et al. Cellular neurothekeoma with possible neuroendocrine differentiation. J Dermatol 1999;26:363-7.  Back to cited text no. 17
Boukovalas S, Rogers H, Boroumand N, Cole EL. Cellular Neurothekeoma: A rare tumor with a common clinical presentation. Plast Reconstr Surg Glob Open 2016;4:e1006.  Back to cited text no. 18


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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