|Year : 2020 | Volume
| Issue : 1 | Page : 59-62
A Rare Entity of S100(−)/cluster of Differentiation 68(+) Jaw Myxoid-Cellular Neurothekeoma: A Case Report and Literature Review
Vida Jawin1, Foo Jen Chun1, Teoh Kean Hooi2, Revathi Rajagopal1
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 Submission||25-Aug-2019|
|Date of Decision||20-Sep-2019|
|Date of Acceptance||27-Nov-2019|
|Date of Web Publication||24-Dec-2019|
Dr Vida Jawin
Department of Pediatric Hematology-Oncology, Level 9, KWKK Block, University Malaya Medical Center, Kuala Lumpur
Source of Support: None, Conflict of Interest: None
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 Feb 19];21:59-62. Available from: http://www.ijpd.in/text.asp?2020/21/1/59/273843
| Introduction|| |
Neurothekeomas are rare, benign soft tissue tumors commonly affecting the adolescents, usually affecting the head, neck, and upper extremities. Histologic variants include myxoid, cellular, and myxoid-cellular (mixed) type., 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].,,,, Among them, only one case has been reported in the pediatric age group. 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.
| Case Report|| |
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 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|| |
Neurothekeomas are rare, benign soft tissue tumors with unknown histogenesis. 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.,, Clinical features include a flesh-colored or slightly erythematous slow-growing papule or nodule of soft consistency.
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). 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.
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.,, The tumor cells are positive for nerve-origin cell markers S100, GFAP, nerve growth factor receptor, collagen type IV, and CD57.,, However, they are negative for epithelial membrane antigen or markers of histiocytic differentiation.,, Interestingly, several authors have reported “S100-negative” myxoid neurothekeoma , [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., 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.,, The diagnosis of cellular neurothekeoma requires immunohistochemistry to identify the fibrohistiocytic markers in addition to histologic appearance., The mixed-type neurothekeoma usually shows overlapping features of both variants with interspersed cellular and myxoid regions. Immunohistochemistry staining is often confusing, revealing irregular or absent reactivity to S100 protein, smooth muscle actin, and positive reaction for fibrohistiocytic differentiation markers.
Strumìa et al. and Henmi et al. reported two cases of S100-negative myxoid neurothekeoma despite distinct myxoid-type by histology., However, fibrohistiocytic marker such as CD68 was not performed in these two cases., 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].,,
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. There is no clear consensus on extent of resection margin, but clear microscopic margin is adequate. 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.,,
| Conclusion|| |
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]