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Sarangi J, Arava S. Verrucous eccrine syringofibroadeoma: An unusual clinical presentation of a rare cutaneous lesion. Indian J Pathol Microbiol 2022;65:500-1
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Sarangi J, Arava S. Verrucous eccrine syringofibroadeoma: An unusual clinical presentation of a rare cutaneous lesion. Indian J Pathol Microbiol [serial online] 2022 [cited 2022 May 5];65:500-1. Available from: https://www.ijpmonline.org/text.asp?2022/65/2/500/343215
Eccrine syringofibroadeoma (ESFA) is an extremely rare tumor that is postulated to arise from the acrosyringium of the eccrine sweat duct and most commonly involves the lower leg and foot of patients in the seventh and eighth decade. It has varied clinical presentations ranging from asymptomatic nodule to verrucous exophytic tumor-like lesion, and histopathological examination is the only way of definitive diagnosis. Till date, there are less than 80 case reports of this entity in the English literature. Based on clinical presentation, ESFA has been divided into five distinct subtypes, namely a) solitary, b) multiple without associated cutaneous findings, c) multiple with hydrotic ectodermal dysplasia, d) nevoid, and e) reactive lesions.,
We would like to highlight a case because of its rarity, unusual clinical presentation as a large verrucous growth with ulcerated and cobblestones appearance, and clinical suspicion of malignancy. Review of the published cases revealed only a single case with a similar clinical appearance.
A 54-year-old male patient presented with an extremely slow-growing verrucous swelling in the right heel area for over 10 years. The lesion was excised with a clear surgical margin and sent for histopathological diagnosis.
The surgically excised specimen measured 50 × 25 × 5 cm with multiple verrucous hypertrophic spongy nodules exhibiting variable pigmentation and varying size from 1 cm to 5 cm. Cut surface of the nodules appeared whitish and densely fibrotic [Figure 1]a.
|Figure 1: Excised specimen showing multiple verrucous hypertrophic spongy nodules. The cut surface is whitish and densely fibrotic (arrow) (a). Microscopic examination of the nodules showed thin anastomosing cords of cuboidal cells extending from the undersurface of the epidermis to the upper dermis embedded in a dense fibrous stroma [b and c; HE, 40 ×]. Occasional ductal luminal structures were seen in the thin anastomosing strands, some of which showed eosinophilic secretions in their lumen (arrow) [d; HE, 400 ×]. On IHC, the ductal structures were positive for epithelial membrane antigen (EMA) [d; inset; 400 ×]|
Sections examined from multiple nodules showed extensive hyperkeratosis, focal parakeratosis, irregular acanthosis, and papillomatosis. In addition, there were prominent thin anastomosing cords of proliferating cuboidal cells extending from the undersurface of the epidermis to the upper dermis with pseudoepitheliomatous hyperplasia–like appearance. These cells exhibited a latticed pattern characteristic of ESFA and were embedded in a dense fibrous stroma with numerous dilated capillaries and sparse inflammatory cell infiltrate composed of lymphocytes, plasma cells, and eosinophils [Figure 1]b, [Figure 1]c. On thorough searching, occasional ductal luminal structures were seen in the thin anastomosing strands (acrosyringium), some of which showed eosinophilic secretions in their lumen. These ductal structures were lined by a single layer of cuboidal cells [Figure 1]d. On immunohistochemical stain, the ductal structures were positive for carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA) confirming the features of an eccrine duct [[Figure 1]d- inset]. Even on extensive sampling of the lesion, areas of dysplasia, atypical mitosis, or necrosis were not noted, ruling out malignant transformation. Based on the characteristic histological picture and the clinical history, a final diagnosis of ESFA was made.
The histopathological differentials considered in this case were verrucous hyperplasia, pseudoepitheliomatous hyperplasia, and squamous cell carcinoma, which were ruled out owing to characteristic anastomosing strands of epithelial cells embedded in a fibrovascular stroma and absence of dysplasia.
Rongioletti et al., have described a case of the mossy leg with eccrine syringofibroadenomatous hyperplasia resembling multiple eccrine syringofibroadeoma in a 46-year-old male patient who presented with bilateral extensive reddish, verrucous, spongy, and malodorous nodules on the legs. They have quoted another case described under the title of elephantiasis nostras verrucosa (lymphoedematous keratoderma or mossy less) with a similar-appearing lesion. This middle-aged psoriatic patient had numerous coalescing malodorous excrescences on bilateral legs. However, the case was interpreted as pseudo carcinomatous hyperplasia probably secondary to venous stasis.
In conclusion, our case illustrates a unique clinical picture of ESFA and calls for consideration of this apparently benign entity in the differential of such exuberant proliferative lesions both by clinicians and pathologists.
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