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| CASE REPORT |
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| Year : 2020 | Volume
: 25
| Issue : 1 | Page : 48-49 |
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Neurothekeoma of the scalp in a child: A rare case
Aditya Pratap Singh1, Maryem Ansari2, Ramesh Tanger1, Arun Kumar Gupta1
1 Department of Pediatric Surgery, SMS Medical College Jaipur, Rajasthan, India
2 Department of Pathology, SMS Medical College Jaipur, Rajasthan, India
| Date of Submission | 24-Jan-2019 |
| Date of Acceptance | 17-Jan-2020 |
| Date of Web Publication | 14-Apr-2020 |
Correspondence Address:
Dr. Aditya Pratap Singh
Department of Pediatric Surgery, SMS Medical College, Near the Mali Hostel, Main Bali Road, Falna, Pali, Jaipur, Rajasthan
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jmgims.jmgims_6_19
Neurothekeoma (NT) is a rare, benign tumor that is derived from peripheral nerve sheath cells. There are less than 300 cases reported in the literature to date. Historically, this tumor has been subclassified as myxoid (classic), mixed, or the cellular type, depending on the amount of myxoid stroma and cellularity. Here, we present a case of NT (mixed type) of the scalp in a 3-year-old female child. The tumor was completely excised. No recurrence was detected in the past 3 months after the local excision.
Keywords: Dermoid, neurothekeoma, scalp, soft tissue
How to cite this article:
Singh AP, Ansari M, Tanger R, Gupta AK. Neurothekeoma of the scalp in a child: A rare case. J Mahatma Gandhi Inst Med Sci 2020;25:48-9 |
| Introduction | |  |
Neurothekeoma (NT) is a rare benign tumor of the nerve sheath, with a distinct histomorphological character. NT is composed of distinct lobules of bland spindle cells separated by fibrous connective tissue with a plentiful myxoid matrix. It is commonly located on the upper extremities or the head and neck and is more common in females.[1] Here, we present a case of scalp swelling in a 3-year-old female child. It was diagnosed as dermoid of the scalp in computed tomography (CT) scan of the head. Histopathology was confirmed it as NT mixed type.
| Case Report | | |
Parent of a 3-year-old female child presented to us with the complaint of the swelling over the scalp for the past 1 year. It was located over the occipital region and measures around 5 cm × 5 cm in size. It was skin covered, soft, mobile swelling. It was increasing in size slowly. Routine blood investigations were within the normal limits, including complete blood counts, renal function, and liver function test. CT of the head showed dermoid of the scalp without any intracranial connection [Figure 1]a. It was excised completely [Figure 1]b. Histopathology showed nodules of cell aggregates, separated by fibrous septa with myxoid changes. Cells were closely spaced, polygonal in shape with pale eosinophilic cytoplasm [Figure 2]. Mitotic figures were noted. On performing immunohistochemistry, cells were focally positive for S-100 and negative for GFAP and CD34. Overall, it was suggested for mixed-type NT. | Figure 2: Histopathology slides (a and b) mitosis in center, (c) alternate cellular and hypocellular myxoid areas
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| Discussion | | |
NTs are slow-growing lesions. They are often solitary and are either asymptomatic or may present as painful, puffy, skin-colored, well circumscribed, and less than 3 cm in diameter nodules. Based on histomorphological appearance and immunohistochemical findings, there are three variants of NT-myxoid (classical or hypocellular), cellular, and mixed type. It is a rare finding in children below the age of 10.[2]
Myxoid (classic and MNT) type has been reported in middle-aged adults and is predominant in females. It is most commonly located on the head, neck, and upper extremities. Classic NTs are considered as a variant of nerve sheath tumors, such as neurofibromas. The MNT is characterized by extremely myxomatous changes and less cellularity with well-circumscribed spindle cells in myxoid matrix and multinucleated giant cells; they stained positively for S100, collagen Type IV, and nerve growth factor receptor and negatively for epithelial membrane antigen or markers of histiocytic differentiation.[3]
The cellular type (CNT) has been observed in younger adults, more commonly in females, and on the head and neck. CNTs have been suggested to be related to plexiform fibrohistiocytic tumor (PFHT) because of the similarities in morphologic and phenotypic features. PFHT is a more aggressive type because of its localization on the trunk and inferior limbs and its higher capacity for infiltration of the subcutaneous fatty tissue.[4]
CNT cells are epithelioid with ample eosinophilic cytoplasm and large “bubbly nuclei” and prominent nucleoli. Atypia and mitotic features are more common in this type.
Myxoid material is usually insufficient and presents only around the individual burrows.
CNTs do not stain S100, collagen type IV, or nerve growth factor receptor, but reactivity with NK1C3 (CD57) and the panmonocyte marker Ki-M1p is positive.[3]
The CNT is a benign tumor because of hypercellularity, the presence of nuclear atypia, and its extension into fat or skeletal muscle, CNT may be mistaken for a malignant tumor, such as sarcoma. These atypical forms of CNT have not shown aggressive behavior.[5] Atypical NT has been described as resembling either benign or malignant melanocytic growths such as malignant melanoma, Spitz nevus, and cellular blue nevus, but melanoma is S100-positive, and CNTs do not stain with antibody to S100 protein.
Mixed-type NT shows the properties of both types.[6] The immunohistochemical features of these cases are confusing because of an irregular or lack of reactivity to S100 and SMA.[5]
A recent article by Rudolph and Schubert[3] underlines the importance of using immunophenotyping rather than light microscopy features alone to classify an NT in the proper category.
Total excision is almost invariably curative, but the tumor showed recurrence in some cases. This recurrence was thought to be secondary to incomplete excision of the primary tumor.[1]
There is no standard value for the resection margins of this tumor, although a few millimeters from a grossly detectable margin may be sufficient. However, due to the varying features of atypical NTs, a punch biopsy to confirm this atypia may be useful before the reconstruction. Bhatia et al.[7] summarize the characteristics of atypical findings as large size (to 6 cm), extension into muscle or fat, vascular invasion, infiltrative borders, high mitotic index, and distinct pleomorphism. If atypical or aggressive features are found in the histological analysis, waiting for permanent margins may be useful.[8] There are no reports in the literature of metastases.
| Conclusion | | |
NTs are infrequent lesions, but when they occur, specific histological analysis and immunophenotyping may be needed if any signs of aggressive behavior are associated with the lesion. Atypical, aggressive tumors may need a two-step approach to wait for permanent margins before embarking on the reconstructive effort.
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.
Acknowledgment
We would like to thank Dr. Neelam Dogra, Anesthesiologist, senior professor, SMS Medical College, Jaipur.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | 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.  |
| 2. | Fetsch JF, Laskin WB, Miettinen M. Nerve sheath myxoma: A clinicopathologic and immunohistochemical analysis of 57 morphologically distinctive, S-100 protein- and GFAP-positive, myxoid peripheral nerve sheath tumors with a predilection for the extremities and a high local recurrence rate. Am J Surg Pathol 2005;29:1615-24. |
| 3. | Rudolph P, Schubert C. Myxoid cellular neurothekeoma. Am J Dermatopathol 2002;24:92-3. |
| 4. | Leclerc-Mercier S, Brousse N, Fraitag S. Is plexiform fibro-histiocytic tumor a deep form of cellular neurothekeoma? J Cutan Pathol 2009;36:1123-5. |
| 5. | Hornick JL, Fletcher CD. Cellular neurothekeoma: Detailed characterization in a series of 133 cases. Am J Surg Pathol 2007;31:329-40. |
| 6. | Yang YW, Shih IH, Huang YH, Kuo TT, Hong HS. Mixed-type neurothekeoma presenting with an unusual clinical appearance of multiple satellite lesions on the back. Dermatol Surg 2005;31:720-2. |
| 7. | Bhatia S, Chu P, Weinberg JM. Atypical cellular neurothekeoma. Dermatol Surg 2003;29:1154-7. |
| 8. | Koumanis DJ, Glickman LT. Facial neurothekeoma in a 10-year-old child. Can J Plast Surg 2007;15:175-7. |
[Figure 1], [Figure 2]
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