.: ORİJİNAL ARAŞTIRMA
Kutanöz Düz Kas Tümörleri: Patolojik Özellikler Açısından 25 Olgunun Retrospektif Analizi
Cutaneous Smooth Muscle Tumors: Retrospective Analysis of 25 Cases in terms of Pathological Features
Turkiye Klinikleri J Med Sci. 2021;41(4):398-409
doi: 10.5336/medsci.2021-83705
Makale Dili: TR
Tam Metin
ÖZET
Amaç: Kutanöz düz kas tümörleri; hamartomatöz, benign ve malign özelliklerde, erektör pili kası, vasküler düz kas veya genital bölgenin özelleşmiş yumuşak dokusundan köken alan nadir tümörlerdir. Bu lezyonların doğru tanımlanması, biyolojik davranışın ve eşlik eden sendromların saptanabilmesi açısından önemlidir. Gereç ve Yöntemler: 2008-2018 yılları arası 10 yıllık dönemde kutanöz düz kas tümörü tanılı 25 olgu klinik ve patolojik özellikleri açısından retrospektif olarak değerlendirilmiştir. Patolojik analizlerde Dünya Sağlık Örgütünün kutanöz ve yumuşak doku tümörleri için önerdiği kriterler kullanılmıştır. Epstein-Barr virüs varlığı, in situ hibridizasyon yöntemi ile değerlendirilmiştir. Bulgular: Serimizin en geniş grubu 11 olgu ile vasküler leiomiyomlar olup 10 olguyu pilar leiomiyom ve 4 olguyu kutanöz leiomiyosarkom oluşturmaktadır. Olguların %52'si erkektir. Lezyonların %44'ü alt ekstremite, %20'si üst ekstremite, %20'si gövde ve %16'sı baş-boyun bölgesindedir. Serimizdeki tüm vasküler leiomiyom-leiomiyosarkomlar ve pilar leiomiyomların %63,6'sı soliter lezyonlardır. Histomorfolojik olarak vasküler leiomiyomların vasküler kanallar ile bağlantılı ve pilar leiomiyomlarında kıl folikülerini çevreleyen düz kas proliferasyonu ile karakterli olduğu gözlenmiştir. Pilar leiomiyom ve vasküler leiomiyom olgularında epiteloid morfoloji, adipoz komponent ve palizatik dizilim nadir görülen değişikliklerdir. Kutanöz leiomiyosarkom olgularından 1'i nodüler paternde diğer 3'ü ise diffüz paterndedir. Selülarite, atipi ve mitotik aktivite nodüler tipte daha belirgindir. Olgularımızda Epstein-Barr virüs varlığı saptanmamıştır. Sonuç: Düz kas tümörleri kutanöz iğsi hücreli neoplazilerin önemli bir grubudur. Çoğu olguda histolojik görünüm tanısaldır. Gerekli durumlarda immünohistokimyasal yöntemle hücre kökeninin belirlenerek mitotik aktivitenin buna göre değerlendirilmesi önemlidir. Normal immüniteye sahip bireylerde Epstein-Barr virüsünün kutanöz düz kas tümörünün patogenezinde rol oynamadığı saptanmıştır.
Anahtar Kelimeler: Deri; düz kas tümörü; vasküler leiomiyom; pilar leiomiyom; leiomiyosarkom
Amaç: Kutanöz düz kas tümörleri; hamartomatöz, benign ve malign özelliklerde, erektör pili kası, vasküler düz kas veya genital bölgenin özelleşmiş yumuşak dokusundan köken alan nadir tümörlerdir. Bu lezyonların doğru tanımlanması, biyolojik davranışın ve eşlik eden sendromların saptanabilmesi açısından önemlidir. Gereç ve Yöntemler: 2008-2018 yılları arası 10 yıllık dönemde kutanöz düz kas tümörü tanılı 25 olgu klinik ve patolojik özellikleri açısından retrospektif olarak değerlendirilmiştir. Patolojik analizlerde Dünya Sağlık Örgütünün kutanöz ve yumuşak doku tümörleri için önerdiği kriterler kullanılmıştır. Epstein-Barr virüs varlığı, in situ hibridizasyon yöntemi ile değerlendirilmiştir. Bulgular: Serimizin en geniş grubu 11 olgu ile vasküler leiomiyomlar olup 10 olguyu pilar leiomiyom ve 4 olguyu kutanöz leiomiyosarkom oluşturmaktadır. Olguların %52'si erkektir. Lezyonların %44'ü alt ekstremite, %20'si üst ekstremite, %20'si gövde ve %16'sı baş-boyun bölgesindedir. Serimizdeki tüm vasküler leiomiyom-leiomiyosarkomlar ve pilar leiomiyomların %63,6'sı soliter lezyonlardır. Histomorfolojik olarak vasküler leiomiyomların vasküler kanallar ile bağlantılı ve pilar leiomiyomlarında kıl folikülerini çevreleyen düz kas proliferasyonu ile karakterli olduğu gözlenmiştir. Pilar leiomiyom ve vasküler leiomiyom olgularında epiteloid morfoloji, adipoz komponent ve palizatik dizilim nadir görülen değişikliklerdir. Kutanöz leiomiyosarkom olgularından 1'i nodüler paternde diğer 3'ü ise diffüz paterndedir. Selülarite, atipi ve mitotik aktivite nodüler tipte daha belirgindir. Olgularımızda Epstein-Barr virüs varlığı saptanmamıştır. Sonuç: Düz kas tümörleri kutanöz iğsi hücreli neoplazilerin önemli bir grubudur. Çoğu olguda histolojik görünüm tanısaldır. Gerekli durumlarda immünohistokimyasal yöntemle hücre kökeninin belirlenerek mitotik aktivitenin buna göre değerlendirilmesi önemlidir. Normal immüniteye sahip bireylerde Epstein-Barr virüsünün kutanöz düz kas tümörünün patogenezinde rol oynamadığı saptanmıştır.
Anahtar Kelimeler: Deri; düz kas tümörü; vasküler leiomiyom; pilar leiomiyom; leiomiyosarkom
ABSTRACT
Objective: Cutaneous smooth muscle tumors are rare tumors of hamartomatous, benign or malignant character, which originate from the erector pili muscle, vascular smooth muscle, or the specialized soft tissue of the genital region. Accurate identification of these lesions is important for determining the biological behavior and accompanying syndromes. Material and Methods: A total of 25 cases of cutaneous smooth muscle tumor diagnosed in a 10-year period between 2008 and 2018 were retrospectively examined for their clinical and pathological characteristics. The criteria proposed by the World Health Organization for cutaneous and soft tissue tumors were used for pathological analyses. The presence of the Epstein-Barr virus was sought by the in situ hybridization technique. Results: The largest group of our series was the vascular leiomyomas comprising 11 cases, followed by pilar leiomyoma with 10 cases and cutaneous leiomyosarcoma with 4 cases. Fifty-two percent of the patients were male. Fortyfour percent of the lesions were located in a lower extremity, 20% in an upper extremity, 20% on the trunk; and 16% in the head & neck region. All vascular leiomyomas-leiomyosarcomas and 63.6% of pilar leiomyomas in our series were solitary lesions. In terms of histomorphology, vascular leiomyomas were found to be linked to vascular channels while pilar leiomyomas were characterized by smooth muscle proliferation surrounding hair follicles. In cases of pilar leiomyomas and vascular leiomyomas, epithelioid morphology, adipose component, and palisade arrangement were rare changes. One of the cutaneous leiomyosarcoma cases had a nodular pattern, and the remaining three had a diffuse pattern. Cellularity, atypia, and mitotic activity were more prominent in the nodular type. Epstein-Barr virus was not detected in our cases. Conclusion: Smooth muscle tumors are an important group of cutaneous spindle cell neoplasms. In most cases the histological appearance is diagnostic. When necessary, it is important to determine the cell origin by immunohistochemical technique and evaluate the mitotic activity accordingly. It has been determined that the Epstein-Barr virus is not involved in the pathogenesis of cutaneous smooth muscle tumor in individuals with normal immunity.
Keywords: Skin; smooth muscle tumor; vascular leiomyoma; pilar leiomyoma; leiomyosarcoma
Objective: Cutaneous smooth muscle tumors are rare tumors of hamartomatous, benign or malignant character, which originate from the erector pili muscle, vascular smooth muscle, or the specialized soft tissue of the genital region. Accurate identification of these lesions is important for determining the biological behavior and accompanying syndromes. Material and Methods: A total of 25 cases of cutaneous smooth muscle tumor diagnosed in a 10-year period between 2008 and 2018 were retrospectively examined for their clinical and pathological characteristics. The criteria proposed by the World Health Organization for cutaneous and soft tissue tumors were used for pathological analyses. The presence of the Epstein-Barr virus was sought by the in situ hybridization technique. Results: The largest group of our series was the vascular leiomyomas comprising 11 cases, followed by pilar leiomyoma with 10 cases and cutaneous leiomyosarcoma with 4 cases. Fifty-two percent of the patients were male. Fortyfour percent of the lesions were located in a lower extremity, 20% in an upper extremity, 20% on the trunk; and 16% in the head & neck region. All vascular leiomyomas-leiomyosarcomas and 63.6% of pilar leiomyomas in our series were solitary lesions. In terms of histomorphology, vascular leiomyomas were found to be linked to vascular channels while pilar leiomyomas were characterized by smooth muscle proliferation surrounding hair follicles. In cases of pilar leiomyomas and vascular leiomyomas, epithelioid morphology, adipose component, and palisade arrangement were rare changes. One of the cutaneous leiomyosarcoma cases had a nodular pattern, and the remaining three had a diffuse pattern. Cellularity, atypia, and mitotic activity were more prominent in the nodular type. Epstein-Barr virus was not detected in our cases. Conclusion: Smooth muscle tumors are an important group of cutaneous spindle cell neoplasms. In most cases the histological appearance is diagnostic. When necessary, it is important to determine the cell origin by immunohistochemical technique and evaluate the mitotic activity accordingly. It has been determined that the Epstein-Barr virus is not involved in the pathogenesis of cutaneous smooth muscle tumor in individuals with normal immunity.
Keywords: Skin; smooth muscle tumor; vascular leiomyoma; pilar leiomyoma; leiomyosarcoma
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