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Here, we report the first cytology findings of the newly characterized entity, palisading adenocarcinoma of the salivary gland, diagnosed in the sublingual gland of a 61-year-old female. The liquid-based cytology showed a moderately cellular aspirate containing three-dimensional clusters and trabeculae of tumor cells of various sizes. The cells had dark ovoid nuclei, finely granular chromatin, inconspicuous to punctate nucleoli, and ample cyanophilic cytoplasm with indistinct cell borders. In conventional smears, the cells displayed frequent crush artifacts and anisonucleosis resembling endocrine-type atypia. The background was clean, devoid of secretions, and contained singly dispersed tumor cells with stripped nuclei. Interestingly, concentrically laminated globules of extracellular matrix surrounded by the tumor cells were identified. Mitotic figures and tumor necrotic debris were absent. The cytologic findings correlated with the histologic findings of the excision specimen. The cytologic differential diagnosis and tumor grading of palisading adenocarcinoma were briefly discussed.
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Multifocal papillary thyroid carcinomas (PTCs) are common and the majority of the tumors harbor mutual BRAF p.V600E mutation. This study aimed to investigate a contemporary series of multifocal PTCs with discordant molecular drivers. Consecutive thyroidectomies diagnosed with multifocal PTCs ≥0.5 cm between 2019 and 2023 were reviewed. Immunohistochemistry (IHC) for BRAF VE1 was performed for all tumors. Cases with discordant BRAF IHC results or morphologic discrepancy were identified, and BRAF IHC-negative tumors were subjected to RAS Q61R IHC and/or targeted RNA next-generation sequencing. A total of 770 patients with a main PTC ≥0.5 cm were identified; 255 (33.1%) had multifocal disease, and 142 (18.4%) had at least another PTC ≥0.5 cm. Among them, 13 cases (9.2%, 13/142) had discordant molecular drivers. Twelve cases had one or more BRAF-positive PTCs accompanied by a BRAF-negative PTC (3 with CCDC6::RET fusion, 1 with NCOA4::RET fusion, 1 with ACBD5::RET fusion, 2 with ETV6::NTRK3 fusion, 1 with TG::FGFR1 fusion, 1 with LMTK2::BRAF fusion, 1 with AGK::BRAF fusion and RAS p.Q61R mutation, 1 with RAS p.Q61R mutation, and 1 without detectable molecular drivers). The last case had tumors with discordant fusion drivers (VIM::NTRK3 and TNS1::BRAF). Most cases showed tumors that were morphologically distinct (92.3%, 12/13) and occurred in the contralateral lobes (76.9%, 10/13). Notably, we identified 4 cases (30.8%) that presented as collision tumors and 6 cases (46.2%) that showed lymph node metastases, including 2 with simultaneous involvement by tumors with discordant molecular drivers, as novel findings. In summary, a subset (9.2%) of multifocal PTCs had discordant molecular drivers and 84.6% of them were a combination of BRAF-positive and kinase gene fusion-associated PTCs, most with distinct morphologies. Almost half of the cases had nodal metastasis and a third of them showed simultaneous involvement by tumors with discordant molecular drivers. The results highlight the clinical importance of identifying such cases, given the potentially different treatments.
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BACKGROUND: Spinal tuberculosis may present in atypical form to involve only the posterior spinal element with relative sparing of the anterior vertebral body and intervertebral disc. Recognition of this unusual pattern is important to avoid delay in diagnosis and treatment. OBSERVATIONS: The authors report a case of a 59-year-old woman with right-sided radiculopathy and motor weakness. Her lumbosacral magnetic resonance imaging showed a large heterogeneous cyst arising from the right L4-5 facet joint. Laminectomy with excision of the cyst was performed. During surgery, the cyst contained cheese-like material that, on histopathological examination, revealed focal aggregates of tuberculous granuloma. Postoperatively, the patient recovered remarkably with no interval development of instability or any deformity. LESSONS: Facet cyst tuberculosis is rare but should be considered in the differential diagnosis in patients coming from endemic regions. Laminectomy with excision of the cyst along with concurrent antitubercular chemotherapy is a safe and durable treatment option in this case.
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OBJECTIVE: Chondroblastoma is an uncommon benign neoplasm of cartilaginous origin usually involving the long bones. The temporal bone is a rare location for this tumor. The clinical profile, optimal medical and surgical management, and outcomes of treatment for temporal bone chondroblastoma remain unknown. METHODS: We performed a systematic review of the SCOPUS, PubMed, and CENTRAL databases for case reports and case series on patients with histopathologically proven temporal bone chondroblastoma. Data on demographics, clinical manifestation, surgical management, adjuvant treatment, and outcome on last follow-up were collected. RESULTS: A total of 100 cases were reported in the literature, including one described in the current study. The mean age of patients was 42.3 years (range, 2-85 years), with a slight male predilection (1.3:1). The most common clinical manifestations were otologic symptoms (e.g., hearing loss [65%], tinnitus, and otalgia) and a palpable mass. Surgical excision was performed in all cases, with gross total excision achieved in 58%. Radiation therapy was performed in 18% of cases, mostly as adjuvant treatment after subtotal excision. There were no deaths at a median follow-up of 2 years. Among the patients with detailed status on follow-up, 58% had complete neurologic recovery, 38% had partial recovery, and 4% had progression of symptoms as a result of tumor recurrence. CONCLUSIONS: Temporal bone chondroblastoma has a distinct clinical profile from chondroblastoma of long bones. Surgery is the mainstay of treatment, and radiation therapy may be given after subtotal excision. Outcomes are generally favorable after treatment.
Asunto(s)
Condroblastoma/terapia , Pérdida Auditiva/fisiopatología , Procedimientos Neuroquirúrgicos , Neoplasias Craneales/terapia , Hueso Temporal/cirugía , Adolescente , Adulto , Distribución por Edad , Anciano , Anciano de 80 o más Años , Audiometría de Tonos Puros , Niño , Preescolar , Condroblastoma/diagnóstico por imagen , Condroblastoma/patología , Condroblastoma/fisiopatología , Dolor de Oído/fisiopatología , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Radioterapia Adyuvante , Recuperación de la Función , Distribución por Sexo , Neoplasias Craneales/diagnóstico por imagen , Neoplasias Craneales/patología , Neoplasias Craneales/fisiopatología , Acúfeno/fisiopatología , Adulto JovenRESUMEN
BACKGROUND: Myasthenia gravis is an autoimmune neuromuscular disorder characterized by the production of abnormal autoantibodies directed against the receptors present in the neuromuscular junction. It has been the standard practice to offer thymectomy in all generalized myasthenia gravis patients despite the lack of robust evidence. OBJECTIVES: The objectives of this study are to describe the clinical profile and differentiate the clinical outcomes of thymectomy versus non-thymectomy and thymomatous versus non-thymomatous myasthenia gravis patients in the Philippine General Hospital. METHODOLOGY: Between 2009 and 2014, a total of 69 postthymectomy and 16 non-thymectomy patient records were successfully retrieved. The demographic characteristics, surgical approach, and histopathologic results were obtained. The clinical outcome after 6 months or 1 year-follow-up was also determined and grouped according to the following: (1) complete remission, (2) pharmacological remission, (3) no clinical change, (4) worsening symptoms, and (5) mortality. RESULTS: Majority of the patients were females (68.0%) with a mean age of 39.8 years and a mean duration of myasthenic symptoms of 21 months. Using the Myasthenia Gravis Foundation of America classification, 54.1% of patients fell under Class II and 48.2% of them presented with generalized weakness. In this study, 60.8% of postthymectomy myasthenia gravis patients had either complete remission or pharmacologic remission compared with 12.5% among non-thymectomy patients (p-value <0.001). No significant difference in the clinical outcome was found between thymomatous and non-thymomatous myasthenia gravis after thymectomy (p-value = 0.29). CONCLUSION: This study showed that both thymomatous and non-thymomatous myasthenia gravis patients who underwent thymectomy had a higher incidence of complete stable remission and pharmacologic remission as compared with myasthenia gravis patients who did not undergo thymectomy.