F Chabbab1, T Metz2, L Saez Beltran2, A Theunis3, B Richert4. 1. Service de dermatologie, CHU Ibn Rochd, 1, rue des Hôpitaux, 20360 Casablanca, Maroc. 2. Département inter-hospitalier de dermatologie-vénéréologie, CHU Brugmann, Saint-Pierre et hôpital universitaire des enfants Reine-Fabiola, université libre de Bruxelles, 4, place Van-Gehuchten, 1020 Bruxelles, Belgique. 3. Département inter-hospitalier de dermatologie-vénéréologie, CHU Brugmann, Saint-Pierre et hôpital universitaire des enfants Reine-Fabiola, université libre de Bruxelles, 4, place Van-Gehuchten, 1020 Bruxelles, Belgique; Département d'anatomie pathologique, Bordet Cancer Institute, université libre de Bruxelles, 121, boulevard de Waterloo, 1000 Bruxelles, Belgique. 4. Département inter-hospitalier de dermatologie-vénéréologie, CHU Brugmann, Saint-Pierre et hôpital universitaire des enfants Reine-Fabiola, université libre de Bruxelles, 4, place Van-Gehuchten, 1020 Bruxelles, Belgique. Electronic address: bertrand.richert@chu-brugmann.be.
Abstract
BACKGROUND: Superficial acral fibromyxoma (SAFM) is a benign soft tissue tumor located in the acral areas, particularly the peri- and sub-ungueal areas. Sub-matricial localisations have not been reported to date. PATIENTS AND METHODS: We report herein the clinical and pathological presentation of three cases of SAFM located solely under the matrix. The patients presented with pseudo-clubbing, onychogryphosis or triangular macrolunula. The histopathological appearance was characteristic. DISCUSSION: SAFM is a slow-growing, skin-colored, firm nodule, located chiefly on the digits or the toes, and especially in the nail area. It may or may not be painful. Microscopically, it presents as a relatively well-circumscribed but unencapsulated dermal tumor, composed of spindle shaped cells integrated in a myxocollagenic matrix, sometimes invading the subcutis. Tumor cells diffusely express CD34. A conservative surgical approach is recommended. Both clinicians and pathologists should be aware of this entity in order to avoid misdiagnosis, which can lead to unwarranted mutilating surgery. CONCLUSION: Sub-matricial localisation of SAFM is extremely rare and may present as pseudo-clubbing, isolated onychogryphosis or a triangular macrolunula. A conservative surgical approach should be recommended.
BACKGROUND: Superficial acral fibromyxoma (SAFM) is a benign soft tissue tumor located in the acral areas, particularly the peri- and sub-ungueal areas. Sub-matricial localisations have not been reported to date. PATIENTS AND METHODS: We report herein the clinical and pathological presentation of three cases of SAFM located solely under the matrix. The patients presented with pseudo-clubbing, onychogryphosis or triangular macrolunula. The histopathological appearance was characteristic. DISCUSSION: SAFM is a slow-growing, skin-colored, firm nodule, located chiefly on the digits or the toes, and especially in the nail area. It may or may not be painful. Microscopically, it presents as a relatively well-circumscribed but unencapsulated dermal tumor, composed of spindle shaped cells integrated in a myxocollagenic matrix, sometimes invading the subcutis. Tumor cells diffusely express CD34. A conservative surgical approach is recommended. Both clinicians and pathologists should be aware of this entity in order to avoid misdiagnosis, which can lead to unwarranted mutilating surgery. CONCLUSION: Sub-matricial localisation of SAFM is extremely rare and may present as pseudo-clubbing, isolated onychogryphosis or a triangular macrolunula. A conservative surgical approach should be recommended.