Alveolar soft part sarcoma (ASPS), a rare soft tissue sarcoma in children and adolescents, carries a poor prognosis. ASPS is an aggressive tumor of controversial histogenesis that, unlike other soft tissue sarcomas, tends to metastasize to the brain. A 9-year-old boy presented to our outpatient clinic in April 2009 with a chief complaint of a large painless mass in the left thigh whose size had increased significantly over the past 10 months. After staging the tumor, we performed open biopsy; the diagnosis was ASPS and he underwent wide local excision. In the course of 4-year follow-up by clinical and imaging studies, there was no evidence of early tumor recurrence or metastasis. Complete surgical resection is the treatment of choice in patients with ASPS.
This is an interesting case of a 53-year-old Caucasian male with a history of recurrent follicular lymphoma (FL) treated with chemotherapy. After 12 years, he developed lymphadenopathy and had an axillary lymph node biopsy which showed transformation to Diffuse Large B-cell lymphoma (DLBCL) with background FL. The patient was treated with chemotherapy and his course was complicated by cellulitis/abscess formation of the right thigh. Computerized Tomography scan showed a large necrotic nodal conglomerate within the right inguinal region measuring 7.8 cm × 5.1 cm and an excisional biopsy was performed. The lymph node architecture was effaced by sheets of large, atypical, pleomorphic cells with open/vesicular chromatin and scattered prominent nucleoli. Focal areas showed residual/recurrent FL, grade I-II. Immunohistochemical stains showed that the large neoplastic cells express CD4, CD68, CD163, BCL-6, lysozyme, CD45 and S-100. The morphologic and immunophenotypic findings are consistent with histiocytic sarcoma (HS). Fluorescence in-situ hybridization studies of the HS showed translocation t(14;18)(q32;q21), a gene rearrangement involving 3q27(BCL6), and gain of 14q32(IgH). The patient's prior FL and DLBCL were shown to have the same translocation t(14;18)(q32;q21). The molecular findings show a common cell lineage between all three diagnoses confirming that FL can transform into DLBCL and transdifferentiate into HS.
Introduction/Objective Hemophagocytic Syndromes are a cluster of disorders related to cytotoxic dysfunction of T/NK-cells and are mainly subdivided into Primary (familial) and Secondary (acquired) forms, with the latter usually linked to patients with viral infections; including EBV, CMV among many others. A myriad of other causes have been associated with hemophagocytic lymphohistiocytosis (HLH), most notably systemic inflammatory conditions; especially Juvenile Rheumatoid Arthritis and hematolymphoid malignancies particularly T/NK-cell lymphomas. Methods/Case Report A previously healthy 7-year-old boy, presented to the ER with fever and a skin rash over both lower limbs of 1 week duration. Two weeks prior he was tested for COVID-19 and was found to be positive. Physical examination further revealed slightly palpable liver and spleen. CBC was done and exhibited pancytopenia, further testing showed elevated LDH, hyperferritinemia and hypertriglyceridemia. However, serological testing for rheumatological conditions was unremarkable. Imaging studies were done and were noncontributory. Subsequently, a bone marrow aspirate and biopsy were done. The bone marrow aspirate showed afew histiocytes engulfing red blood cells and nuclear debris (hemophagocytic cells), complete trilineage maturation and normal M:E ratio of 3:1. Trephine biopsy was hypocellular for age and estimated at about 70%, composed of myeloid and erythroid precursors with various degrees of maturation. Megakaryocytes were adequate in number and showed normal morphology. Extensive histiocytic infiltration as highlighted by CD68 immunostain and focal phagocytosis were identified. CD34 highlighted <5% blasts, PAS special stain showed no fungal elements and no fibrosis was evident by Reticulin special stain. The background was devoid of lymphoid aggregates or granulomas. Stainable iron stores were depleted. No sideroblasts were identified. The patient was treated with corticosteroid and showed marked improvement and was discharged after 3 days. Results (if a Case Study enter NA) NA Conclusion Hemophagocytic lymphohistiocytosis can be a critical sequela of COVID-19 infection. Suggested mechanisms include impaired/delayed T-cell response and elevated levels of several inflammatory cytokines. Clinical suspesion is important in the diagnosis of these cases. Further study of this correlation is needed as we explore clinical sequelae of COVID-19 infection.
We present the case of a female infant who initially presented to our institution at approximately 7 months of age with a several month history of vomiting, failure to thrive, and developmental delay. Past medical history was remarkable for prematurity (born at 30 weeks gestation secondary to preterm premature rupture of the membranes) and neonatal intensive care unit stay complicated by intubation and feeding difficulties. Physical exam was remarkable for hepatosplenomegaly. Laboratory tests revealed hypercalcemia (17.9 mg/dL; reference range = 8.8-10.8 mg/dL), mild hyponatremia (137 mmol/L; 138-145 mmol/L), elevated thyroid-stimulating hormone (6.34 µIU/mL; 0.47-4.68 µIU/ml) with normal free T4 (1.09 ng/dL; 0.65-1.85 ng/dL), metabolic acidosis (HCO 3 16 mmol/L;
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