Selective Venous Sampling Prompting Unilateral Oophorectomy in an Adolescent With PCOS and Markedly Elevated Testosterone.

BACKGROUND: For adolescents with suspected polycystic ovary syndrome (PCOS) and severely elevated testosterone concentrations, imaging is recommended to assess for neoplasm. Selective venous sampling (SVS) can be considered when imaging is nondiagnostic. CASE: An adolescent female treated for PCOS had a peak testosterone of 344 ng/dL (11.9 nmol/L). Imaging did not localize a mass. SVS implicated the right ovary as the source of hyperandrogenism. Following laparoscopic right oophorectomy, pathology excluded a neoplasm and confirmed PCOS. She subsequently had rapid and persistent improvement in her hyperandrogenism. SUMMARY AND CONCLUSION: Striking testosterone elevation can occur with adolescent PCOS. SVS is a tool for localizing the source of severe hyperandrogenism, yet unilaterality is not always diagnostic of a neoplasm. Unilateral oophorectomy could nonetheless be therapeutic for severe PCOS.

Perinucleolar Compartment (PNC) Prevalence as an Independent Prognostic Factor in Pediatric Ewing Sarcoma: A Multi-Institutional Study.

The perinucleolar compartment (PNC) is a small nuclear body that plays important role in tumorigenesis. PNC prevalence correlates with poor prognosis and cancer metastasis. Its expression in pediatric Ewing sarcoma (EWS) has not previously been documented. In this study, we analyzed 40 EWS tumor cases from Caucasian and Hispanic patients for PNC prevalence by immunohistochemical detection of polypyrimidine tract binding protein and correlated the prevalence with dysregulated microRNA profiles. EWS cases showed staining ranging from 0 to 100%, which were categorized as diffuse (≥77%, n = 9, high PNC) or not diffuse (<77%, n = 31) for low PNC. High PNC prevalence was significantly higher in Hispanic patients from the US (n = 6, p = 0.017) and in patients who relapsed with metastatic disease (n = 4; p = 0.011). High PNC was associated with significantly shorter disease-free survival and early recurrence compared to those with low PNC. Using NanoString digital profiling, high PNC tumors revealed upregulation of eight and downregulation of 18 microRNAs. Of these, miR-320d and miR-29c-3p had the most significant differential expression in tumors with high PNC. In conclusion, this is the first study that demonstrates the presence of PNC in EWS, reflecting its utility as a predictive biomarker associated with tumor metastasis, specific microRNA profile, Hispanic ethnic origin, and poor prognosis.

Normal or reactive minor cell populations in bone marrow and peripheral blood mimic minimal residual leukemia by flow cytometry.

BACKGROUND: Measurable residual disease (MRD) is a strong independent poor prognostic factor for acute leukemia. Multiparameter flow cytometry (FCM) is a commonly used MRD detection method. However, FCM MRD detection is not well standardized, and the interpretation is subjective. There are normal/reactive minor cell populations in bone marrow (BM) and peripheral blood (PB), which could be confused with MRD. METHODS: The FCM data of 231 BM and 44 PB pediatric samples performed in a recent 15-month period were retrospectively reviewed. These samples were from 56 B-lymphoblastic leukemia (B-ALL) patients, 11 T-lymphoblastic leukemia (T-ALL) patients, 28 acute myeloid leukemia (AML)/myelodysplastic syndrome (MDS) patients, 44 cytopenia/leukocytosis patients, and five patients with mycosis fungoides. RESULTS: There were over 10 normal or reactive minor cell populations identified with certain phenotypes mimicking MRD of acute leukemia. These mimickers included CD19+ NK cells, CD22+ basophils, CD22+ dendritic cells (DCs), and plasma cells for B-ALL MRD; CD4/8 double-negative T cells, CD4/8 double-positive T cells, cytoplasmic CD3+ NK cells, CD2- T cells, CD7- T cells, CD5- gamma delta T cells, CD56+ NKT cells for T-ALL MRD; CD33+ NK cells, CD117+ NK cells, basophils, plasmacytoid DCs, non-classical monocytes, CD56+ and/or CD61+ monocytes for AML MRD. CONCLUSIONS: These data confirm the presence of a variety of normal/reactive minor cell populations that could mimic MRD of acute leukemia by FCM. Recognizing these MRD mimickers is important for correct FCM MRD interpretation.