RESUMO
BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is an exceedingly rare and aggressive tumor, with limited literature on its management. Herein, we present our series of surgically managed craniospinal MPNSTs, analyze their outcomes, and review the literature. METHODS: We retrospectively reviewed surgically managed primary craniospinal MPNSTs treated at our institution between January 2005 and May 2023. Patient demographics, tumor features, and treatment outcomes were assessed. Neurological function was quantified using the Frankel grade and Karnofsky performance scores. Descriptive statistics, rank-sum tests, and Kaplan-Meier survival analyses were performed. RESULTS: Eight patients satisfied the inclusion criteria (4 male, 4 female). The median age at presentation was 38 years (range 15-67). Most tumors were localized to the spine (75%), and 3 patients had neurofibromatosis type 1. The most common presenting symptoms were paresthesia (50%) and visual changes (13%). The median tumor size was 3 cm, and most tumors were oval-shaped (50%) with well-defined borders (75%). Six tumors were high grade (75%), and gross total resection was achieved in 5 patients, with subtotal resection in the remaining 3 patients. Postoperative radiotherapy and chemotherapy were performed in 6 (75%) and 4 (50%) cases, respectively. Local recurrence occurred in 5 (63%) cases, and distant metastases occurred in 2 (25%). The median overall survival was 26.7 months. Five (63%) patients died due to recurrence. CONCLUSIONS: Primary craniospinal MPNSTs are rare and have an aggressive clinical course. Early diagnosis and treatment are essential for managing these tumors. In this single-center study with a small cohort, maximal resection, low-grade pathology, young age (< 30), and adjuvant radiotherapy were associated with improved survival.
Assuntos
Neoplasias de Bainha Neural , Neurofibromatose 1 , Neurofibrossarcoma , Humanos , Masculino , Feminino , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Neurofibrossarcoma/patologia , Neoplasias de Bainha Neural/cirurgia , Neoplasias de Bainha Neural/diagnóstico , Estudos Retrospectivos , Resultado do Tratamento , Neurofibromatose 1/patologiaRESUMO
Gender diversity, especially pertaining to transgender and gender-diverse (TGD) populations, is often stigmatized. A small but not insignificant number of adults in the United States identify as TGD, including transgender, nonbinary, and other gender identities than cisgender. Accessing health care remains a significant challenge for TGD individuals because many health care systems adhere to a gender binary model and many TGD individuals experience negative interactions when interfacing with health care. There is also a scarcity of literature addressing their unique health care needs, limiting our current understanding of breast cancer risks and screening recommendations for TGD patients. This article reviews important considerations when providing care to TGD patients. It covers background information on gender identity and sexuality, explores gender-affirming care, discusses histopathologic findings of breast biopsy specimens, examines breast cancer risks, and presents current breast cancer screening recommendations for TGD patients. Education on TGD breast cancer risks and screening and creating a standardized screening protocol for TGD patients who may receive gender-affirming care through hormonal and surgical therapies could help improve their health care equity and access.
Assuntos
Neoplasias da Mama , Detecção Precoce de Câncer , Pessoas Transgênero , Humanos , Neoplasias da Mama/patologia , Neoplasias da Mama/diagnóstico , Neoplasias da Mama/diagnóstico por imagem , Feminino , Pessoas Transgênero/psicologia , Masculino , Mama/diagnóstico por imagem , Mama/patologia , Mamografia , Assistência à Saúde Afirmativa de GêneroRESUMO
BACKGROUND: Malignant triton tumors (MTTs) are a rare and aggressive type of malignant peripheral nerve sheath tumor identified histologically by focal rhabdomyoblastic differentiation. OBSERVATIONS: A 37-year-old female with a prior history of Hodgkin lymphoma presented with acute-onset confusion, cognitive deficits, and weakness. Brain magnetic resonance imaging revealed a hemorrhagic intracranial mass later confirmed to be a malignant triton tumor. The patient underwent two resections and several courses of chemoradiation for multiple tumor recurrences and metastases. Unfortunately, despite extensive treatment, she died 2 years after initial presentation from complications of this tumor. Her overall survival (OS) of 26.7 months was double that reported in historical cohorts (OS â¼13 mos). LESSONS: The diagnosis and treatment of central nervous system MTTs are difficult and require a multidisciplinary team of neurosurgeons, radiologists, and oncologists. Histopathological analysis is required for confirmation of diagnosis. Gross-total resection of tumor and adjuvant radiation therapy have been shown to give patients the highest rates of survival and improved outcomes. Further studies and clinical trials are warranted to investigate the efficacy of chemotherapeutic agents like temozolomide, bevacizumab, and abemaciclib.
RESUMO
BACKGROUND: Pediatric brainstem abscesses are rare entities that account for 1% of all brain abscesses and, when diagnosed, constitute a neurosurgical emergency. OBSERVATIONS: A previously healthy 11-year-old male presented with several days of worsening headache, confusion, and ataxia. Brain magnetic resonance imaging (MRI) revealed a midbrain and pons lesion. The patient subsequently had a rapid neurological decline with loss of consciousness and brainstem function. Follow-up MRI revealed significant enlargement of the brainstem lesion with extension into the pons, midbrain, and thalamus, with greater concerns for an abscess rather than a tumor or an inflammatory process. He was taken for an emergent stereotactic aspiration of the abscess, and broad-spectrum antibiotics were initiated. He had neurological improvement, which subsequently declined 5 days later with brain MRI revealing an increase in the brainstem abscess, which required a second stereotactic aspiration. After rehabilitation, he made a significant neurological recovery. LESSONS: Pediatric brainstem abscesses are rare pathologies, and a high index of suspicion is needed in patients presenting with a brainstem lesion mimicking tumor but with rapid neurological decline despite no other evidence of infection or infectious/inflammatory markers. Stereotactic aspiration is required for large lesions to target the antibiotic treatment and as an adjunct to broad-spectrum antibiotics.