ABSTRACT
Chordomas are rare locally invasive malignant bone tumors arising from remnants of embryonic notochord. Dedifferentiated chordoma (DC), a rare subtype, is characterized by the presence of a sarcomatous component in conventional chordoma (CC) which may arise de novo or as a malignant transformation of previously treated chordoma. The presence of dedifferentiation warrants a poor prognosis due to distant metastasis and recurrences. De novo DCs pose a diagnostic challenge especially in small biopsies and at metastatic sites. Here, we report the case of a 45-year-old female presenting with a long history of backache and constipation, finally diagnosed as DC. Radiological as well as histomorphological pictures of the tumor posed diagnostic challenges because they can mimic other tumors occurring in a similar location. We found this case worth reporting as de novo DC is rarely reported in the literature and it has the potential to pose diagnostic as well as therapeutic challenges.
ABSTRACT
A 68-year-old man was referred to us with clinical and bone marrow (BM) features compatible with aplastic anemia. The correct diagnosis, hypoplasia of the BM coexisting with multiple myeloma, became apparent after noting rouleaux in the peripheral blood (PB) and approximately 50% plasma cells in the touch imprint of one of the two BM biopsies done. As standard therapy was precluded, the patient was put on dexamethasone but died within 4 days. This first case of the coexistence of untreated myeloma with aplastic BM shows that even apparently straightforward hypoplasia seen on the BM biopsy should be interpreted in conjunction with the PB smear and the BM touch imprint findings. Among other things, the BM biopsy and imprint should be repeated if the PB has findings such as rouleaux that do not fit with straightforward aplastic anemia. The combination of myeloma and BM aplasia precludes standard therapy and is rapidly fatal.