Dermatomyositis as paraneoplastic syndrome of peritoneal and ovarian relapse after long-term complete remission in patient with metastatic bilateral breast cancer [Dermatomiozitis kao paraneoplastički sindrom peritonealnog i ovarijalnog relapsa nakon dugog perioda potpune remisije u bolesnice s metastatskim bilateralnim rakom dojke]

Murgić, Jure and Prpić, Marin and Kirac, Iva and Camino-Varela, Adriana-Maria and Bolanča, Ante and Kusić, Zvonko (2012) Dermatomyositis as paraneoplastic syndrome of peritoneal and ovarian relapse after long-term complete remission in patient with metastatic bilateral breast cancer [Dermatomiozitis kao paraneoplastički sindrom peritonealnog i ovarijalnog relapsa nakon dugog perioda potpune remisije u bolesnice s metastatskim bilateralnim rakom dojke]. Collegium Antropologicum, 36 (1). pp. 325-329. ISSN 0350-6134

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Abstract

Dermatomyositis is a rare disease characterised by inflammatory muscle affection and characteristic cutaneous changes. When occuring in a patient with cancer, dermatomyositis may indicate recurrence or progression and poor outcome. Herein, the treatment of metastatic breast cancer, metastatic pattern, characteristics of long-term survivors, and link between dermatomyositis and breast cancer are discussed and the literature reviewed. We report a 57-year old female patient with metastatic bilateral breast cancer whose ovarian and peritoneal relapse after long-term remission was disclosed by occurence of paraneoplastic dermatomyositis. The patient previously had a 15-year long disease free-period after primary treatment for breast cancer before onset of pulmonary dissemination. Following antracycline-based chemotherapy, the complete remission lasting another 15 years was accomplished. Dermatomyositis had been resolved upon induction of second-line taxane-based chemotherapy. After completion of six cycles of gemcitabine and paclitaxel chemotherapy, check-up revealed further progression. The patient subsequently underwent six cycles of third-line CAP chemotherapy (cyclofosfamide, doxorubicine, cisplatin) but disease progressed and oral capecitabine chemotherapy was initiated. The patient received four cycles of capecitabine followed by further vast progression and finally expired following massive pulmonary embolism. Our case stresses the need of thorough staging and check-up when dermatomyositis arises in patients with breast cancer, regardless of previous stable long-term complete remission. Furthermore, we believe that treatment with curative intent in young patients with metastatic breast cancer, who have good performance statuses and no comorbidities is required, because it is more likely to produce long-term complete remission. However, following disease relapse a poor outcome can be expected.

Abstract in Croatian

Dermatomiozitis je rijetka bolest karakterizirana upalnim promjenama u mišićima i karakterističnim kožnim promjenama. Kada se pojavi u bolesnika s rakom, dermatomiozitis može upućivati na povrat ili progresiju maligne bolesti te često ukazuje na loš ishod. U ovom radu opisali smo povezanost između dermatomiozitisa i raka dojke, te raspravili mogućnosti liječenja metastatskog raka dojke, kao i značajke bolesnica koje smatramo izliječenima – tzv. »long-term survivors«. Prikazujemo slučaj 57-godišnje bolesnice s metastatskim bilateralnim karcinomom dojke u koje je pojava paraneoplastičkog dermatomiozitisa u podlozi razotkrila recidiv osnovne bolesti u jajnicima te po peritoneumu. Bolesnica je prethodno imala 15 godina dugo razdoblje bez znakova bolesti po završetku primarnog liječenja, nakon čega je nastupila plućna diseminacija. Po provedenoj kemoterapiji baziranoj na antraciklinima nasupio je kompletan odgovor (remisija) koja je trajala 15 godina, nakon čega je nastupila prethodno opisana diseminacija u područje jajnika te peritoneuma. Dermatomiozitis se povukao po uvođenju druge linije kemoterapije bazirane na taksanima. Po kompletiranju šest ciklusa kemoterapije gemcitabinom i paklitakselom, obrada je ukazala na daljnju progresiju bolesti. Bolesnica je potom primila šest ciklusa treće linije kemoterapije po CAP protokolu (ciklofosfamid, doksorubicin, cisplatin), no bolest je progredirala i započeta je peroralna kemoterapija kapecitabinom. Bolesnica je primila četiri ciklusa terapije kapecitabinom, nakon čega je nastupila daljnja opsežna progresija te je bolesnica konačno preminula pod slikom masivne plućne embolije. Ovaj slučaj ukazuje na potrebu temeljitog „staginga“ i kliničke obrade u slučaju pojave dermatomiozitisa u bolesnica s rakom dojke, bez obzira na prethodnu trajnu i stabilnu kompletnu remisiju. Nadalje, držimo da je u mladih bolesnica s metastatskim rakom dojke koje su dobrog općeg stanja i nemaju komorbiditeta potrebno liječenje s kurativnom namjerom, jer je moguće da se takvim liječenjem postigne dugotrajna kompletna remisija. Ipak, ako konačno dođe do povrata bolesti (relapsa), očekuje se loš ishod.

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