Acral lesions in tuberous sclerosis complex: Insights into pathogenesis

Aldrich, Capt Shelley L.; Hong, Chien Hui; Groves, Leslie R.; Olsen, Cara; Moss, Joel; Darling, Thomas N.

doi:10.1016/j.jaad.2009.08.042

Cited by 61 publications

(51 citation statements)

References 31 publications

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“…Molecular genetic data are lacking, but rare cases have been associated with Rb aberrations. 9,10,12,13 Various techniques have been proposed for excising difficult lesions. 11 Fibro-osseous pseudotumor of digits Fibro-osseous pseudotumor of digits may become absorbed by the umbrella term myositis ossificans, to which it is microscopically nearly identical.…”

Section: Fibroma Of Tendon Sheathmentioning

confidence: 99%

Acral manifestations of soft tissue tumors

Paral

Petronic‐Rosic

2017

Clinics in Dermatology

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This group of biologically diverse entities is united by topographic localization to the hands and feet. Categorizing tumors by body site narrows the differential into a short list of possibilities that can facilitate accurate and rapid diagnosis. The goal of this review is to provide a practical approach to soft tissue tumors of acral locations for clinicians, pathologists, and researchers alike. What ensues in the following text is that tight coupling of the clinical picture and histopathologic findings should produce the correct diagnosis, or at least an abbreviated differential. The salient clinicopathologic, immunohistochemical, and molecular features are presented alongside current treatment recommendations for each entity.

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Section: Fibroma Of Tendon Sheathmentioning

confidence: 99%

Acral manifestations of soft tissue tumors

Paral

Petronic‐Rosic

2017

Clinics in Dermatology

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“…Periungual and subungual fibromas, or Koenen tumors, commonly occur in patients with tuberous sclerosis, with an incidence of 15% to 52% in this population. 24 Onset typically occurs between the second and fifth decades 24 ; there have been reports of incidence as high as 88% in patients over age 30. 9 A recent study of 76 patients (all female) with tuberous sclerosis found 61 patients to have ungual fibromas; periungual tumors were more common than tumors in the subungual region.…”

Section: Soft Tissue Neoplasms Fibromamentioning

confidence: 99%

“…Some patients described preceding trauma before the development of ungual fibromas; however, the exact etiology remains unknown. 24 Large subungual fibromas present as pink papules originating from under the nail plate, which may cause lifting of the nail plate with visible hyperkeratosis (Fig. 8).…”

Section: Soft Tissue Neoplasms Fibromamentioning

confidence: 99%

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Benign Subungual Tumors

Willard

Cappel

Kozin

et al. 2012

The Journal of Hand Surgery

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“…Su localización en la zona periungueal puede provocar anomalías en la lámina ungueal Las presiones del calzado favorecen el crecimiento del tamaño de las lesiones de las uñas, siendo los traumatismos los que desencadenan la formación de estos tumores 12 . No existe una etiopatogenia clara de esta patología, si bien el factor traumático puede influir en el crecimiento de la lesión 14,15 .…”

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Tratamiento quirúrgico del Tumor de Koënen: expresión podológica de la Esclerosis Tuberosa

Coheña‐Jiménez

Jiménez

Rodríguez

et al. 2014

Rev. Int. Cienc. Podol.

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Los autores declaran no tener ningún tipo de interés económico o comercial. ReSuMeNLos Tumores de Koënen son manifestaciones cutáneas de la Esclerosis Tuberosa, que es de afectación sistémica y en ocasiones con localización en el pie. El conocimiento de este tipo de afecciones dermatológicas es muy importante para un tratamiento correcto y por este motivo un enfoque interdisciplinario es esencial para que el diagnóstico sea preciso y precoz. En la Historia Clínica es importante realizar una valoración de los antecedentes familiares, realizar una exploración de la piel y un estudio neurológico. Se presenta un caso con clínica compatible con la presencia de Tumor de Koënen. El diagnostico diferencial es esencial para plantear el tratamiento y conseguir un buen pronóstico y pronta curación. Conclusiones. Se plantea el tratamiento quirúrgico como técnica de elección pese a la elevada tasa de recidivas que presenta dicho tratamiento. La resección quirúrgica de la lesión permite el diagnóstico anatomopatologico. Palabras clave: esclerosis tuberosa; tumor de koënen; tratamiento podológico; tratamiento quirúrgico. ABStRACtThe Koenen tumors are cutaneous manifestations of tuberous sclerosis, which is of systemic involvement and sometimes with location in the foot. The knowledge of this type of dermatological is very important for proper treatment and for this reason an interdisciplinary approach is essential for the accurate and early diagnosis. In the clinical history is important to make an assessment of family history, perform a scan of the skin and a neurological study. We report a case with symptoms consistent with the presence of tumor Koenen. The differential diagnosis is essential for planning treatment and get a good outcome and speedy recovery. Conclusions. Surgical treatment is proposed as a technique of choice despite the high rate of recurrence presents such treatment. Surgical resection of the lesion allows the pathological diagnosis.

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Acral lesions in tuberous sclerosis complex: Insights into pathogenesis

Abstract: Background-Patients with tuberous sclerosis complex (TSC) are predisposed to developing ungual fibromas and other acral lesions.

Cited by 61 publications

References 31 publications

Acral manifestations of soft tissue tumors

Acral manifestations of soft tissue tumors

Benign Subungual Tumors

Tratamiento quirúrgico del Tumor de Koënen: expresión podológica de la Esclerosis Tuberosa

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