Giant periungual and subungual fibromas (Koenen’s tumor) of tuberous sclerosis complex

Reddy, Indukooru Subrayalu; Somani, V K

doi:10.25259/ijdvl_1194_20

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“…Substantial involvement of the nail apparatus may lead to local pain, recurrent infections, and difficulty to perform daily activities as seen in the presented case. Multiple giant KTs affecting all digits are uncommon [16][17][18]. In some patients, these lesions may become keratotic and are often misdiagnosed and mistreated as periungual warts and fibrokeratomas [16][17][18][19].…”

Section: Discussionmentioning

confidence: 99%

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Giant Koenen Tumors as the Presenting and Only Clinical Sign of Tuberous Sclerosis Complex in a 56-Year-Old Man and Its Surgical Management

Olvera-Rodríguez

Garibay

Domínguez‐Cherit

2022

Skin Appendage Disord

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Introduction: Tuberous sclerosis complex (TSC) is a genetic multisystem disorder with prominent skin involvement. Multiple ungual fibromas, also known as Koenen tumors (KTs), are one of the major diagnostic criteria and occur approximately in 50% of patients with TSC. They are disfiguring, painful, and challenging to treat as they frequently recur. There are 2 previous cases in the literature of KTs as the only clinical sign of TSC. Case Presentation: A 56-year-old male patient with intellectual disability presented with a history of ungual growths that had gradually increased in size and number for 20 years. The diagnosis of KTs was established on clinical grounds, without the requirement of histological confirmation. No additional clinical evidence of TSC was found. Complete surgical excision of the tumors located in the proximal nail matrix, periungual folds, and nail bed of each toe was successfully performed. Discussion: The association between KTs and intellectual disability with TSC supported the clinical diagnosis. To date, there is no standard first-line treatment for KTs. Complete surgical excision up to the proximal base of the tumor offers satisfactory results in terms of functionally and esthetic appearance and could be considered the preferred therapeutic modality for patients with severe functional and cosmetic impairment. Prompt recognition of the many signs and symptoms associated with TSC is important to achieve early diagnosis, preventing further complications.

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Section: Discussionmentioning

confidence: 99%

“…Multiple giant KTs affecting all digits are uncommon [16][17][18]. In some patients, these lesions may become keratotic and are often misdiagnosed and mistreated as periungual warts and fibrokeratomas [16][17][18][19]. Squamous-cell carcinoma should be also considered in the differential diagnosis [16].…”

Section: Discussionmentioning

confidence: 99%