RESUMO
BACKGROUND: Metastatic neuroendocrine carcinoma (NEC) of bone marrow is uncommon. Here, we report a case of bone marrow metastatic NEC with an unknown primary site. CASE SUMMARY: A 73-year-old Chinese woman was admitted to our hospital because marked chest distress and asthma lasting 1 d on March 18, 2018. She was initially diagnosed with pulmonary infection, cardiac insufficiency, thrombocytopenia and severe anemia. Following treatment with antibiotic therapy, diuresis and blood transfusion, the patient's symptoms greatly improved. After bone marrow examinations, the patient was diagnosed with bone marrow metastatic NEC, bone marrow necrosis (BMN) and secondary myelofibrosis (MF). Further imaging workup did not show the primary tumor, we presumed that the primary site might regress spontaneously or merely be unexplored due to lack of positron emission tomography with gallium peptide. Everolimus (10 mg/d) was added to the treatment and the best supportive and symptomatic therapies were also administered. Unfortunately, the patient's condition continued to deteriorate and she died on May 15, 2018. CONCLUSION: Bone marrow invasion of NEC is rare and our patient who suffered from bone marrow metastatic NEC as well as secondary BMN and MF had an extremely poor prognosis. Bone marrow biopsy plays an important role in the diagnosis of solid tumors invading bone marrow.
RESUMO
BACKGROUND: Whether hepatocellular carcinoma (HCC) patients with hypersplenism can benefit from splenectomy is unclear. This study aimed at exploring the efficacy and safety of concurrent splenectomy for HCC patients with hypersplenism. METHODS: PubMed, EMBASE and Web of Science databases were systematically searched to compare data on the combination of hepatectomy or transhepatic arterial infusion (TAI) with splenectomy (the splenectomy group) with data on hepatectomy or TAI alone (the non-splenectomy group) for the treatment of HCC with hypersplenism. Prospective clinical trials or retrospective cohort studies from inception to May 10, 2020 were considered eligible for this analysis. The relevant outcomes, including patients' demographics, clinicopathologic characteristics, perioperative indices and long-term outcomes, were independently extracted by two investigators. Publication bias for overall survival (OS) and disease-free survival (DFS) was qualitatively assessed by funnel plots and quantitatively evaluated by Begg's and Egger's tests. RESULTS: Nine retrospective studies including 1,650 patients were analyzed. Short-term outcomes suggested that the incidence rate of postoperative complications, including portal or splenic vein thrombosis [odds ratio (OR) =26.28, P<0.001] and pancreatic injury (OR =14.89, P=0.001), was significantly higher in the splenectomy group, whereas the perioperative mortality rate was similar between the splenectomy and non-splenectomy groups (P=0.541). Long-term outcomes indicated that the occurrence of variceal re-hemorrhage (OR =0.31, P<0.001) and tumor progress or recurrence (OR =0.62, P=0.001) was markedly reduced for patients who underwent splenectomy, while the long-term mortality rates were not statistically different between the two groups (P=0.087). The prognostic evaluation revealed that the OS and DFS were comparable between the splenectomy and non-splenectomy groups [for OS: hazard ratio (HR) =0.77, 95% confidence interval (CI): 0.53-1.13; for DFS: HR =0.87, 95% CI: 0.63-1.19]. Funnel plots suggested an HRs symmetric distribution for OS and DFS. Begg's and Egger's tests confirmed that there was no significant HR publication bias for OS and DFS. CONCLUSIONS: Due to the significant progress in surgical techniques and perioperative care, concomitant splenectomy should be considered as an optional treatment for some HCC patients with hypersplenism.
RESUMO
BACKGROUND: The Janus kinase 2 (JAK2) V617F mutation is common in patients with breakpoint cluster region-Abelson1 (BCR-ABL1)-negative myeloproliferative neoplasms, including polycythemia vera, essential thrombocythemia and primary myelofibrosis, but is rarely detected in BCR-ABL1-positive chronic myeloid leukemia (CML) patients. Here, we report a CML patient with both a BCR-ABL1 rearrangement and JAK2 V617F mutation. CASE SUMMARY: A 45-year-old Chinese woman was admitted to our department with a history of significant thrombocytosis for 20 d. Color Doppler ultrasound examination showed mild splenomegaly. Bone marrow aspiration revealed a karyotype of 46, XX, t(9;22)(q34;q11.2) in 20/20 metaphases by cytogenetic analysis, rearrangement of BCR-ABL1 (32.31%) by fluorescent polymerase chain reaction (PCR) and mutation of JAK2 V617F (10%) by PCR and Sanger DNA sequencing. The patient was diagnosed with CML and JAK2 V617F mutation. Following treatment with imatinib for 3 mo, the patient had an optimal response and BCR-ABL1 (IS) was 0.143%, while the mutation rate of JAK2 V617F rose to 15%. CONCLUSION: Emphasis should be placed on the detection of JAK2 mutation when CML is diagnosed to distinguish JAK2 mutation-positive CML and formulate treatment strategies.
