Case Report

Atypical Fibroxanthoma of the Scalp in a Young Woman: A Case Report

Khadija Elboukhari1*, Asmae Rasso1, Sara Elloudi1, Zakia Douhi1, Hanane Baybay1, Fatima Zahra Mernissi1 and Afaf Amarti²

¹ Department of Dermatology, University Hospital of Fez, Morocco

²Al Azhar Laboratory of Anatomy pathology, Fez, Morocco

Submission: March 23, 2020;Published: April 13, 2020

*Corresponding author: Khadija Elboukhari, Department of Dermatology, University Hospital of Fez, Morocco

How to cite this article: Khadija E, Asmae R, Sara E, Zakia D, Hanane B, Fatima Z M, Afaf A. Atypical Fibroxanthoma of the Scalp in a Young Woman: A Case Report. JOJ Dermatol & Cosmet. 2020; 2(4): 555595. DOI: 10.19080/JOJDC.2020.02.555595

Abstract

Atypical fibroxanthoma, is a uncommon tumor of fibrohistiocytic mesenchymal origin, it occurs on the sun-damaged skin of elderly men. The clinical presentation is a solitary cutaneous nodule. The diagnosis is made by histology and immunohistochemistry. The large excision is the mainstay therapy and the recurrence is possible. We report a case of atypical Fibroxanthoma occurring in the scalp of a young woman.

Keywords: Atypical Fibroxanthoma; Mesenchymatous; Scalp

Abbreviations: AFX: Atypical Fibroxanthoma

Introduction

AFX is a rare parenchymatous tumor, it is considered by some authors as a low-grade sarcoma [1]. This cutaneous neoplasm originates from fibrohistiocytic mesenchymatous tissue [2]. It affects elderly men, in photodamaged area, such are head and neck. This tumor is characterized by a favorable prognosis and a rare risk of metastasis [3].

Case Report

A young woman of 28 years old, was referred for a solitary nodule of the scalp, evolving since 02 months, without pain or pruritus, the dermatological examination showed a nodule of the frontal scalp of 1cm size (Figure 1), erythematous and slightly ulcerated, painless. It was superficial, without subjacent involvement . The rest of the somatic examination did not show any abnormalities. In front of this clinical presentation we evocated a proliferating trichilemmal cyst, a squamous cell carcinoma, an achromic melanoma, our patient benefited from a biopsy that showed a proliferation of pleomorphic atypical spindle-shaped cells (Figure 2). In Immunochemistry, the HMB 45 was negative, eliminating a chromic melanoma (Figure 3), the Cytokeratin was negative too eliminating a Squamous cell carcinoma (Figure 4). While the fixation of CD67 (Figure 5) confirmed our diagnostic of AFX. The patient benefited from cervical ultrasonography that didn’t show any ganglionary involvement, then the tumor was excised with 1 cm margins. The first control was without abnormalities, the scars didn’t show recurrence signs. A regular follow up is conducted for our patient (Figure 6).

Discussion

AFX is a rare distinctive cutaneous neoplasm of fibrohistiocytic mesenchymal origin. The oncogenetic background of this tumor demonstrates C-T mutation in P53 and TERT promoter genes [4,5], which explain the location in actinically damaged skin. Moreover, the occurrence of this tumor in patients with Xeroderma Pigmentosum supports this hypothesis. Radiation, burn traumatism and maybe the risk factor of the occurrence of this tumor [6]. Its pathogeny is still unclear, and it may originate from myofibroblasts or apparent fibroblast cells [7]. Although this tumor affects elderly men, there is a small subgroup of affected younger patients [1] as was the case of our patient. Clinically, AFX appears as a solitary ulcerated nodule, of 1,5-2cm size, in a sundamaged area, mimicking a squamous cell carcinoma or basal cell carcinoma [1] as was the case of our patent. Histological features of this tumor include the presence of dense cellularity with haphazardly ranged, atypical spindle-shaped cells just beneath the epidermis, extending to the dermis and hypodermis [2]. However, this description is not specific to AFX, it can be found in the other spindle cell tumors, especially the pleomorphic dermal sarcoma which share a clinical, histological and either immunostain characteristics with AFX [8]. The management of this tumor is a total excision, while the marges are still unprecise. The risk of recurrence is variable through the reported cases, and it is ranging from 3 to 20% [9,10].

Conclusion

AFX is a rare neoplasm that may occur in young patients and should be evocated in front of a solitary nodule in the sun-exposed area.

References

1. Marcet S (2008) A typical fibroxanthoma/malignant fibrous histiocytoma. Dermatologic Therapy 21: 424-427.
2. Péron J, Depaepe L, Decroze D, Jaouen A, Dalle S, et al. (2014) Fibroxanthome atypique. Annales de Dermatologie et de Vénéréologie 141(12): 786–789.
3. Soleymani T, Tyler Hollmig S (2017) Conception and Management of a Poorly Understood Spectrum of Dermatologic Neoplasms: Atypical Fibroxanthoma, Pleomorphic Dermal Sarcoma, and Undifferentiated Pleomorphic Sarcoma. Curr Treat Options in Oncol 18: 50.
4. Lai K, Harwood CA, Purdie KJ, Ravi N, Arron ST et al. (2017) Genomic anal-ysis of atypical fibroxanthoma. PLoS One 12(11): e0188272.
5. Helbig D, Quaas A, Mauch C, Jan B, Elke B, et al. (2017) Copy numbervariations in atypical fibroxanthomas and pleomor-phic dermal sarcomas. Oncotarget 8(65): 109457-109467.
6. Lorizzo LJ, Brown MD (2011) A typical fibroxanthoma: are view of the literature. DermatolSurg 37(2): 146-157.
7. Weedon D (2010) 34-Tumors and tumor-like proliferations of fibrous and related tissues. In: Weedon D (Ed.), Weedon’s Skin Pathology (3^rd Edⁿ). Churchill Livingstone, Edinburgh, pp 809-844.e29.
8. Dilek FH, Akpolat N, Metin A, Ugras S (2000) Atypical fibroxanthomaof the skin and the lower lip in xeroderma pigmentosum.BrJ Dermatol 143(3): 618 -620.
9. Soleymani T, Aasi SZ, Novoa R, Hollmig, ST (2019) Atypical Fibroxanthoma and Pleomorphic Dermal Sarcoma. Dermatologic Clinics 37(3): 253-259.
10. Huether MJ, Zitelli JA, Brodland DG (2001) Mohs micrographic surgery for the treatment of spindle cell tumors of the skin. Journal of the American Academy of Dermatology 44(4): 656-659.