Lymphomas
Contributions
Type: Publication Only
Background
Hemophilic pseudotumor is characterized by slowly expanding, encapsulated mass. It develops as a result of extra-articular musculoskeletal hemorrhage, in 1-2% of hemophiliacs. Hemophilia treatment has significantly improved, bringing the opportunity for age-related morbidities development, including malignancies. Sometimes after imaging diagnostics, surgery is necessary for differential diagnostics.
Aims
To present hemophiliac patient with large tumor mass, initially suspected for hemophilic pseudotumor. Pathohistological finding of tumor biopsy revealed HCC metastasis with HCV infection. We discuss treatment option for such patient.
Methods
Single case report
Results
Hemophilia B with F IX 1% was diagnosed at the age of 15. Forty years later, our patient was diagnosed with HCV infection and he received subsequent antiviral therapy. After four years, following abdominal CT finding (liver necrotic lesions) and presence of elevated serum alpha-fetoprotein (AFP, 174ng/ml), HCC diagnosis was established. Trans-arterial chemoembolization (TACE) was opted as treatment choice but prior to intervention, right forearm painful swelling occurred. Since MRI finding of right arm and shoulder showed large tumor mass in humeroscapular region with severe osteolytic lesions of the right humerus, hemophilic pseudotumor was highly suspected. F IX concentrate substitusion was applied but with no obvious therapy response. Subsequently, tumor biopsy was performed and it revealed HCC metastasis. PET scan finding confirmed this metastasis as solitary. Several treatment options were discussed. Since HCC is known to be poor radiotherapy responder, and it would only be applied to metastasis, we excluded this treatment. Second generation tyrosin kinases inhibitors that would have a certain degree of systemic effect were not available in our country. Radical surgery that included right hand amputation was also excluded, due to a very high perioperative risk and present comorbiditiy (Diabetes mellitus type 2). Patient was subsequently treated with palliative therapy. Five months later, lethal outcome occurred, after severe bleeding in humeroscapular region.
Summary
A leading cause for HCC in hemophilia is HCV chronic infection. Extra hepatic metastases occur in approximately half of HCC patients, and in our case tumor biopsy revealed very unusual HCC metastasis. If HCV infection is diagnosed it has to be treated. Due to the high risk for HCC in hemophilia, regular ultrasonography should be performed. When pseudotumor is suspected, HCC possibility should also be investigated. HCC should be surgically treated in its early stage.
Keyword(s): Cancer, Hemophilia B, Hepatitis C virus
Session topic: Publication Only
Type: Publication Only
Background
Hemophilic pseudotumor is characterized by slowly expanding, encapsulated mass. It develops as a result of extra-articular musculoskeletal hemorrhage, in 1-2% of hemophiliacs. Hemophilia treatment has significantly improved, bringing the opportunity for age-related morbidities development, including malignancies. Sometimes after imaging diagnostics, surgery is necessary for differential diagnostics.
Aims
To present hemophiliac patient with large tumor mass, initially suspected for hemophilic pseudotumor. Pathohistological finding of tumor biopsy revealed HCC metastasis with HCV infection. We discuss treatment option for such patient.
Methods
Single case report
Results
Hemophilia B with F IX 1% was diagnosed at the age of 15. Forty years later, our patient was diagnosed with HCV infection and he received subsequent antiviral therapy. After four years, following abdominal CT finding (liver necrotic lesions) and presence of elevated serum alpha-fetoprotein (AFP, 174ng/ml), HCC diagnosis was established. Trans-arterial chemoembolization (TACE) was opted as treatment choice but prior to intervention, right forearm painful swelling occurred. Since MRI finding of right arm and shoulder showed large tumor mass in humeroscapular region with severe osteolytic lesions of the right humerus, hemophilic pseudotumor was highly suspected. F IX concentrate substitusion was applied but with no obvious therapy response. Subsequently, tumor biopsy was performed and it revealed HCC metastasis. PET scan finding confirmed this metastasis as solitary. Several treatment options were discussed. Since HCC is known to be poor radiotherapy responder, and it would only be applied to metastasis, we excluded this treatment. Second generation tyrosin kinases inhibitors that would have a certain degree of systemic effect were not available in our country. Radical surgery that included right hand amputation was also excluded, due to a very high perioperative risk and present comorbiditiy (Diabetes mellitus type 2). Patient was subsequently treated with palliative therapy. Five months later, lethal outcome occurred, after severe bleeding in humeroscapular region.
Summary
A leading cause for HCC in hemophilia is HCV chronic infection. Extra hepatic metastases occur in approximately half of HCC patients, and in our case tumor biopsy revealed very unusual HCC metastasis. If HCV infection is diagnosed it has to be treated. Due to the high risk for HCC in hemophilia, regular ultrasonography should be performed. When pseudotumor is suspected, HCC possibility should also be investigated. HCC should be surgically treated in its early stage.
Keyword(s): Cancer, Hemophilia B, Hepatitis C virus
Session topic: Publication Only