RESUMO
Phyllodes tumor (PT) is considered a rare fibroepithelial tumor. Very few series have been reported in children and adolescents. Based on histopathological features, it can be classified as benign, borderline, or malignant, with the latter having a more aggressive clinical behavior. We report the case of a 10-year-old female who began with an asymptomatic mobile right breast mass. An initial fine needle biopsy (FNB) concluded fibroadenoma (FA). Months later, the mass kept growing, with the appearance of pain and nipple discharge. Benign PT was demonstrated in a new biopsy. A total mastectomy was performed. The post-surgical histopathological examination was compatible with a borderline PT. The patient is now symptom-free and with no signs of relapse. Not all breast masses in the pediatric or adolescent age bracket are FA. Attention is warranted when the clinical behavior does not follow the usual outline. PT has to be considered as a possible diagnosis and treated accordingly.
RESUMO
Anaplastic large cell lymphoma (ALCL) is children's most common mature T-cell neoplasm. The majority is positive for anaplastic lymphoma kinase (ALK). Initial presentation as a soft-tissue pelvic mass without nodal involvement is rare and can be easily misdiagnosed. We report a case of a 12-year-old male presenting with pain and movement restriction in the right extremity. Computed tomography (CT) scan revealed a solitary pelvic mass. Initial biopsy examination concluded rhabdomyosarcoma. After developing pediatric multisystemic inflammatory syndrome due to coronavirus disease 2019 (COVID-19), central and peripheral lymph node enlargement appeared. New cervical adenopathy and pelvic mass biopsies were performed. Immunohistochemistry concluded an ALK-positive ALCL with a small-cell pattern. The patient was treated with brentuximab-based chemotherapy and eventually improved. Differential diagnosis of pelvic masses in children and adolescents must include ALCL. An inflammatory trigger may promote the appearance of a typical nodal disease, previously absent. Attention is warranted during histopathological examination to avoid diagnostic errors.
RESUMO
Acute lymphoblastic leukemia (ALL) is an aggressive hematological neoplasm typically more common in children than adults. More prolonged remissions and a potential cure can be achieved if allogeneic hematopoietic stem cell transplantation (allo-HSCT) is performed. Outcomes after allo-HSCT vary significantly among patients, and multiple factors contribute to these outcomes. Isolated extramedullary relapse (iEMR) after allo-HSCT is rare. We present the case of a 43-year-old man who was diagnosed with Philadelphia chromosome-negative (Ph-neg), B-cell ALL and underwent haploidentical allo-HSCT because of high-risk features at diagnosis. One year later, he was admitted to the hospital with facial and peripheral edema, proteinuria, elevated serum creatinine levels, and hypertension. Renal biopsy was performed immediately. Renal infiltration of TdT+ leukemic cells was detected by immunohistochemistry. Bone marrow aspiration, lumbar puncture, and computed tomography (CT) scans were performed to identify other sites of possible relapse. No other sites were identified, and an extramedullary isolated renal relapse was diagnosed. Intensive re-induction with chemotherapy was not possible because of the coronavirus disease 2019 (COVID-19) infection. Six weeks later, a medullary relapse was noted. Medullary infiltration of B-cell ALL after allo-HSCT has a historically poor prognosis; however, iEMR appears to have a better overall prognosis. The optimal treatment for renal iEMR is still a matter of debate.
RESUMO
Congenital thrombotic thrombocytopenic purpura (cTTP) is an inherited disease that is sometimes fatal in early childhood. cTTP is similar to idiopathic thrombotic thrombocytopenic purpura (iTTP); both are characterized by varying levels of thrombocytopenia, microangiopathic hemolytic anemia (MAHA), and end-organ damage secondary to occlusion of the microvasculature. cTTP is caused by a partial or total deficiency or loss of function of ADAMTS-13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13). We report the case of a 33-year-old woman who was mistakenly diagnosed with primary immune thrombocytopenia (ITP) during childhood. The patient was referred to our center with dyspnea, fatigue, fever, and jaundice with no clinical bleeding. Laboratory features were compatible with MAHA; ADAMTS-13 activity was at 0%, with negativity for ADAMTS-13 antibodies. We concluded the final diagnosis was cTTP. The triggering factor identified for MAHA was a double infection: central venous catheter bacterial infection and atypical pneumonia. After 7 days of treatment with antibiotics and ongoing total plasma exchange (TPE), the patient responded favorably. Our patient received fresh frozen plasma (FFP) infusion once every 2 weeks, and prophylactic voriconazole remained under control at the time of writing. As demonstrated in this case, effective treatment of the trigger cause helps reduce the need for continuous FFP exposure and controls the MAHA.