Malignant peripheral nerve sheath tumor with hemophilic syndrome and bone marrow fibrosis:A rare case report

BACKGROUND Malignant schwannoma is a rare tumor in the peripheral nervous system,accounting for approximately 5%to 10%of systemic soft tissue sarcomas.Especially,malignant schwannoma occurring in the broad ligament of the uterus with hemophilic syndrome and bone marrow fibrosis is extremely rare in clinical practice.Here,we report the first case of an patient diagnosed with malignant peripheral nerve sheath tumor(MPNST)of the broad ligament of the uterus with hemophilic syndrome and bone marrow fibrosis,and share our reference clinical diagnosis and treatment experience.CASE SUMMARY A patient was diagnosed with MPNST of the uterus harboring hemophilic syndrome and bone marrow fibrosis.She received combination,and repeated imaging revealed further encountered rare complications(hemophilia syndrome and bone marrow fibrosis)after two cycles of chemotherapy.Thereafter,combined treatment with pazopanib,gemcitabine,and dacarbazine was initiated.Unfortunately,the patient succumbed to death at hospital after two weeks.CONCLUSION This report firstly provided reference clinical practice for a patient with MPNST of the uterus harboring hemophilic syndrome and bone marrow fibrosis.Our case raises a reminder about the tolerance and safety of combination therapy,especially in young women.

Acute Panmyelosis with Myelofibrosis: A Rare Subtype of Acute Myeloid Leukemia

Acute panmyelosis with myelofibrosis (APMF) is a subtype of acute myeloid leukemia (AML) classified among the category of “AML, not otherwise specified” in the WHO 2016 classification of hematopoietic tumors. It is a rare, fatal hematological neoplasm that is characterized by acute onset of cytopenias and bone marrow fibrosis in the absence of splenomegaly or fibrosis related morphological changes in the red blood cells. The difficulty of diagnosis and management explains why APMF is rarely reported in Africa. We report here the case of a 30-year-old man who presented with dizziness, palpitations and dyspnea. Diagnosis of APMF was retained on bone marrow histology and immunohistochemistry which showed bone marrow fibrosis and high cellularity with majority of myeloid blast cells. The patient was treated by low dose cytarabine monotherapy 30 mg/m2 per week. At 3 months of treatment, the patient was transfusion-independent, with normalization of hemoglobin and platelets counts. However, the death occurred after 8 months. This case highlights the diagnosis specificity and management of AMPF, knowing the number of potential differential diagnoses and difficulties of its therapeutic management.