Tumoral calcinosis presenting as a soft tissue mass in a 16 year old patient

Autor: Marija Milković Periša, Sanda Šitić, Irena Veliki Dalić, Ljubica Luetić Cavor, Ilija Guteša, Sven Seiwerth
Jazyk: angličtina
Rok vydání: 2017
Předmět:
Zdroj: Libri Oncologici : Croatian Journal of Oncology
Volume 45
Issue 1
ISSN: 2584-3826
0300-8142
Popis: Background: Tumoral calcinosis is a rare clinicopathological condition characterized by periarticular tumor-like calcium deposits most commonly found around major joints, especially the hips, shoulders and elbows. Tumoral calcinosis is not primarily a bone-or cartilage-forming lesion, but it can closely simulate osteocartilaginous tumors. These lesions are predominantly seen during the first two decades of life and may be multicentric or bilateral. Laboratory analysis frequently shows high serum phosphate but normal serum calcium levels. A 16 year old male presented with a huge mass in the left hip which had been growing for a month. MR detected large tumor mass in the left gluteal region with possible infi ltration of m.gluteus maximus. Soft tissue mass was surgically resected, measuring 24 cm in diameter. Histologically, it was composed of multiple large cystic spaces with nodules of amorphous calcifi ed deposits surrounded by multinucleated giant cells and mononuclear infi ltrates. The lesion was poorly circumscribed with infi ltration into surrounding striated muscle. Tumoral calcinosis is an uncommon and benign condition that generally occurs as a complication of trauma or renal dialysis, and is rarely seen in familial and sporadic cases. It can occur in a variety of clinical sett ings: primary normophosphatemic, primary hyperphosphatemic and secondary tumoral calcinosis occuring along with disorders that are capable of producing soft tissue calcifi cation. Histologically, tumoral calcinosis is characterized by amorphous calcifi ed deposits in the background of granulomatous appearance with multinucleated giant cells and other infl ammatory cells. The diff erential diagnosis is broad and includes all tumoral calcinosis-like lesions that lead to abnormal dystrophic or metastatic calcium deposition in soft tissues. Surgical excision is the primary treatment.
Tumorska kalcinoza je rijetko kliničko-patološko stanje obilježeno periartikularnim kalcificiranim depozitima nalik tumorima najčešće smještenim u području kuka, ramena i lakta. Tumorska kalcinoza nije primarna bolest koštano-hrskavičnog sustava, iako svojim obilježjima nalikuje i upućuje na tumor koštano-hrskavičnog sustava. Izraslina se obično pojavljuje multicentrično i bilateralno u prva dva desetljeća života. Laboratorijska analiza učestalo pokazuje povećanu razinu fosfata i normalnu razinu kalcija u serumu. Lječeni dječak (16 god) imao je veliku tvorbu u području lijevog kuka koja je rasla mjesecima. MR je prikazala izraslinu s mogućom infiltracijom velikog glutealnog mišića. Kirurški je odstranjena neoštro ograničena tvorba najvećeg promjera 24 cm koja je infiltrirala strijatni mišić. Histološki je bila građena od brojnih velikih cističnih prostora s okruglastim kalcificiranim nakupinama okruženim multinuklearnim orijaškim stanicama i mononuklearnim upalnim infi ltratom.Tumorska kalcinoza je rijetko, benigno stanje koje se uglavnom očituje kao komplikacija traume ili bubrežne dijalize, a rijetko se viđa i u obiteljskom i u sporadičnom obliku. Može se očitovati u nekoliko kliničkih oblika: primarna normofosfatemična, primarna hiperfosfatemična i sekundarna tumorska kalcinoza. Neovisno o obliku uvijek je prisutan poremećaj s nakupljanjem kalcifikata u mekom tkivu. Histološki se vide amorfni kalcificirani depoziti okruženi granulomatoznom upalom s multinuklearnim orijaškim i mononuklearnim upalnim stanicama. Diferencijalna dijagnoza obuhvaća široki spektar promjena koje uključuju pretjerano distrofično i metastatsko nakupljanje kalcija u mekom tkivu. Kirurška ekscizija je izbor liječenja.
Databáze: OpenAIRE
Externí odkaz: