Advances in Clinical and Experimental Medicine

Title abbreviation: Adv Clin Exp Med
JCR Impact Factor (IF) – 1.736
5-Year Impact Factor – 2.135
Index Copernicus  – 168.52
MEiN – 70 pts

ISSN 1899–5276 (print)
ISSN 2451-2680 (online)
Periodicity – monthly

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Advances in Clinical and Experimental Medicine

2006, vol. 15, nr 6, November-December, p. 1113–1119

Publication type: review article

Language: English

Inflammatory Myofibroblastic Tumor – Pathological and Clinical Aspects

Inflammatory myofibroblastic tumor – patologia i klinika

Maria Pomorska1,, Marta Rzeszutko1,

1 Chair and Department of Pathological Anatomy, Silesian Piasts University of Medicine, Wrocław, Poland

Abstract

The recently described inflammatory myofibroblastic tumor is an uncommon lesion of extrapulmonary and pulmonary tissue with an unpredictable clinical course, occasional recurrence, and rare malignant transformation. The cause and pathogenesis are unclear, but it has been postulated that a subset of IMT is characterized by the expression of altered anaplastic lymphoma kinase (ALK) protein, mostly resulting from rearrangements of the ALK gene, such as the TPM3−ALK, TPM4−ALK, and CLTC−ALK fusion genes. IMT is a distinctive mesenchymal lesion composed of spindle cells displaying morphological features of myofibroblasts admixed with considerable numbers of inflammatory cells. Immunohistochemistry is of little help in diagnosis. IMT may have a relatively low mitotic rate without atypical mitotic figures, areas of necrosis and stromal hyalinization, nuclear atypia and, above all, without “intravascular” tumor spread. The biological behavior of myofibroblastic proliferation cannot always be confidently predicted from histological features (although metastases are distinctly uncommon), and follow−up is often recommended. Surgical resection remains the treatment of choice. Radiotherapy has been described as an adjunct in unresectable lesions and complete resection of recurrences is recommended. An aggressive surgical approach is necessary as there is a limited role for other adjunctive modalities. The report demonstrates the unusual features of IMT with regard to both clinical and morphological aspects.

Streszczenie

Inflammatory myofibroblastic tumor (IMT), jest niedawno rozpoznaną, rzadko spotykaną zmianą o płucnym i pozapłucnym umiejscowieniu, cechującą się nieprzewidywalnym przebiegiem klinicznym, obserwowanymi nawrotami i rzadko występującą transformacją nowotworową złośliwą. Patogeneza tej choroby nie jest znana, lecz uważa się, że u części IMT występuje zmieniona ekspresja kinazy anaplastycznego chłoniaka (ALK) w wyniku rearanżacji genu ALK i jego fuzji z innymi genami, takimi jak: tropomiozyna 3 TPM3−ALK, tropomiozyna 4 TPM4−ALK i klarytyna CLTC−ALK. IMT jest oddzielną jednostką chorobową o charakterystycznych cechach morfologicznych. Guzy te składają się z wrzecionowatych komórek wykazujących cechy miofibroblastów z domieszką komórek zapalnych w różnych proporcjach. Reakcje immunohistochemiczne nie są decydujące w ich rozpoznaniu. IMT cechuje się niskim indeksem mitotycznym, brakiem atypowych figur podziałowych, martwicy, atypii jądrowej, a przede wszystkim nie szerzy się drogą naczyń krwionośnych. Biologia rozrostów miofibroblastycznych, oceniana na podstawie cech histologicznych, pozostaje jednak nieprzewidywalna i konieczna jest dalsza obserwacja chorych po zabiegu operacyjnym. Leczeniem z wyboru jest resekcja chirurgiczna zmiany, a radioterapię stosuje się jako leczenie wspomagające zmian nieoperacyjnych. Zalecana jest także całkowita resekcja zmian nawrotowych. Agresywne postępowanie chirurgiczne jest niezbędne z powodu braku innych skutecznych metod leczenia. Praca przedstawia charakterystyczne cechy IMT zarówno pod względem klinicznym, jak i morfologicznym.

Key words

myofibroblastoma, plasma cell granuloma, inflammatory myofibroblastic tumor

Słowa kluczowe

guz miofibroblastyczny, ziarniniak plazmatycznokomórkowy, zapalny guz miofibroblastyczny

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