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Arborizing vessels under dermoscopy: A case of cellular neurothekeoma instead of basal cell carcinoma

  • Author(s): Aydingoz, Ikbal Esen
  • Mansur, Ayse Tulin
  • Dikicioglu-Cetin, Emel
  • et al.
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Arborizing vessels under dermoscopy: A case of cellular neurothekeoma instead of basal cell carcinoma
Ikbal Esen Aydingoz MD, Ayse Tulin Mansur MD, Emel Dikicioglu-Cetin MD
Dermatology Online Journal 19 (3): 5

Acibadem University, School of Medicine Istanbul, Turkey

Abstract

Neurothekeoma is a slow-growing, benign tumor of nerve sheath origin. Herein we present a 62-year-old female who presented with a 5-month history of a nodule that had shown a slight enlargement. She had a diagnosis of non-Hodgkin lymphoma for 10 years for which she had received multiple sessions of chemotherapy and radiotherapy. Cutaneous examination showed a well-defined, firm, 2 cm, pink-red nodule of the right supraclavicular area, which showed thick and arborizing vessels under dermoscopy. A diagnosis of cellular neurothekeoma was made after histopathologic examination with immunohistochemistry. Thick and arborizing vessels have been described as the dermoscopic hallmark of nodular and cystic basal cell carcinoma. In the past, hydradenoma and intraepidermal poroma have been defined as dermoscopic mimics of basal cell carcinoma because of the characteristic appearance of arborizing vessels. With this report a neurogenic tumor has been added to this list.


A 62-year-old female presented with a 5-month history of a nodule that had showed a slight enlargement. Her past medical history was noteworthy for Non-Hodgkin lymphoma (NHL) in 2001 for which she had received chemotherapy and radiotherapy. She had had a recurrence of NHL at the base of the skull in 2008 and was given a second session of radiotherapy. In 2009 she experienced cavernous sinus thrombosis associated with multiple and extensive thrombi. Later in 2010, occlusive hydrocephalus was diagnosed, which was treated by ventriculoperitoneal shunt insertion. Cutaneous examination showed a well-defined, firm, 2 cm, pink-red hard nodule on the right supraclavicular area. Close inspection disclosed prominent vessels. The background skin showed normal color and consistency (Figure 1). Dermoscopy showed only thick and arborizing vessels on the surface of the nodule (Figure 2).


Figure 1Figure 2
Figure 1. Clinical appearance of the lesion

Figure 2. Dermoscopy showing arborizing vessels (Heine x10)

Histopathology revealed a dermal mesenchymal tumor with superficial subcutaneous extension. It was composed of nests and fascicles of a whorling arrangement of spindle cells. In addition, there were focally clear appearing cells and scattered osteoclast-like giant cells. The intervening stroma showed myxoid foci and some inflammatory cells (Figure 3). Immunohistochemically, lesional cells stained positive with vimentin, CD68, CD10, and NSE. The cells were negative for Pan-CK, CD34, SMA, HMB-45, and S-100. A diagnosis of cellular neurothekeoma was made.


Figure 3
Figure 3. (3a) General view of the cellular neurothekeoma (H&E x 40). (3b) The tumor composed of nests and fascicles of a whorling arrangement of spindle cells (H&E x 100). (3c) Intervening stroma with some myxoid foci shown by an arrow. (H&E x200). (3d) Positive immunohistochemical stains with NSE (H&E x200)

Neurothekeoma was first described in 1969 by Harkin and Reed as “nerve sheath myxoma” [1]. It is a slow-growing, benign tumor of nerve sheath origin, which occurs mostly on the upper extremities or the head and neck in young females [1]. Mucosal lesions comprise about 10 percent of the cases [1, 2]. There are three histologic variants of the tumor. The classical or myxoid type is characterized by a myxomatous plexiform pattern. The cellular type shows a predominantly cellular, fascicular pattern. Mixed cases demonstrate a combination of both [1, 3]. Clinical findings of cellular neurothekoma are non-specific. Usually it presents as a cupuliform, firm, flesh-colored or hyperpigmented asymptomatic papulo-nodular growth, which rarely exceeds 10 mm. It has been reported to grow slowly with a benign course. In some of the cases capillaries have been noted on its surface. Thus, Benbenisty [3], Lopez-Cepeda [4], and Cecchi [5] have all reported telangiectasias on the surface of their patients’ lesions. Neurothekeoma is a rare tumor and demoscopic findings have not been reported before. Thick and arborizing vessels on the surface of this tumor can even be seen with the naked eye; these are features described as the dermoscopic hallmark found in nodular, and cystic BCC [6]. The more common nodular type of BCC is mainly observed on the head and neck [7] similar to neurothekeomas. In non-pigmented skin tumors vessels may be the only dermoscopic structure observable, providing valuable information for diagnosis. Large diameter stem vessels branching irregularly into terminal vessels appearing bright red and focused under dermoscopy have been described as common in BCC and recognition of this has been reported to increase the diagnostic accuracy of BCC diagnosis [8-13]. This specific morphology of arborizing vessels has shown high diagnostic accuracy with a sensitivity of 96.1 percent and specificity of 90.9 percent [14]. In another dermoscopic study based on 531 skin lesions, arborizing vessels were seen in 82.1 percent of the cases of BCC with a PPV of 94.1 percent [11]. The most common vascular pattern showed arborizing vessels with a percentage of 60.7, significantly more frequent in nodular BCCs [13]. In their recent study examining non-pigmented BCCs, Altamura et al. concluded arborizing vessels to be reliable and robust parameters to diagnose BCC [12].

Clinical and dermoscopic mimics of basal cell carcinoma have been reported before. Hydradenoma and intraepidermal poroma showing arborizing vessels are the examples in this contex [15]. Now, a neurogenic tumor has been added to this list.

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