Introduction. Primary angiosarcoma of the breast (PAB)
is a very rare tumor and accounts for 0.04% of all breast
malignant tumors and most commonly occurs in young
women. Kasabach-Merritt syndrome (KMS) is described
as consumption coagulopathy with thrombocytopenia,
and without adequate therapy almost certainly leads to a
very fast lethal outcome. The literature contains only a
few cases of PAB associated with thrombocytopenia or
with KMS and there are no clear defined protocols for
the treatment of these patients, which requires the
presentation of as many cases as possible. Case report.
We presented a rare case of 60-year-old postmenopausal
woman with metastatic PAB grade III associated with a
picture like KMS (thrombocytopenia and anemia without
the coagulation factor disorder with massive bleeding in
the tumor). Mastectomy was performed without the extirpation of the axillary region. After the surgery, improvement with anemia and thrombocytopenia was noticed. One month after the surgery, supportive and
symptomatic therapy was administered, as well as bishosphonate therapy, but with temporary improvement.
Deplasmated erythrocytes and methylprednisolone were
added during the another hospital stay, but prominent
symptoms of general weakness along with the progression od thrombocytopenia were noted. Fibrinogen and
coagulation factors were within reference values all the
time. The patient died four months after the surgery.
Conclusion. PAB in postmenopausal women is a very
rare tumor, and may be associated with anemia and
thrombocytopenia without other laboratory parameters
for KMS. Anemia and thrombocytopenia are refractory
to standard treatment protocols, and also significantly
reduces the quality of life of these patients.