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BACKGROUND: Malignant triton tumors (MTTs) comprise a subgroup of malignant peripheral nerve sheath tumors (MPNSTs) that exhibits rhabdomyosarcomatous differentiation and follow an aggressive course. MTTs are primarily located along peripheral nerves. Cases of MTTs in the abdominal wall have not been reported. MTT has a poorer prognosis than classic MPNSTs, and accurate diagnosis necessitates a keen understanding of the clinical history and knowledge of its differential diagnosis intricacies. Treatment for MTTs mirrors that for MPNSTs and is predominantly surgical. CASE SUMMARY: A 49-year-old woman presented with a subcutaneous mass in her lower abdominal wall and a pre-existing surgical scar that had grown slowly over 3-4 months before the consultation. She had previously undergone radical hysterectomy and concurrent chemo-radiotherapy for cervical cancer approximately 5 years prior to the consultation. Abdominal computed tomography (CT) showed a 1.3 cm midline mass in the lower abdomen with infiltration into the rectus abdominis muscle. There was no sign of metastasis (T1N0M0). An incisional biopsy identified sporadic MTT of the lower abdomen. A comprehensive surgical excision with a 3 cm margin inclusive of the peritoneum was executed. Subsequently, the general surgeon utilized an approach akin to the open peritoneal onlay mesh technique. The patient underwent additional treatment with an excision shaped as a mini-abdominoplasty for the skin defect. No complications arose, and annual follow-up CTs did not show signs of recurrence or metastasis. CONCLUSION: An abdominal MTT was efficaciously treated with extensive excision and abdominal wall reconstruction, eliminating the need for postoperative radiotherapy.
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Background: Malignant triton tumors (MTT) are subtype of malignant peripheral nerve sheath tumor (MPNST) which develop from Schwan cells of peripheral nerves or within neurofibromas, and shows rhabdomyoblastic differentiation. It is a rare soft tissue tumor with poor prognosis. Objective: We report a case of Malignant Triton Tumor (MTT) arising in the right shoulder in a 46 year old male patient presented to our Musculoskeletal Oncology Clinic at Royal Rehabilitation center at King Hussein Medical Center during June 2018. Case presentation: The patient was complaining of an 8 months long progressive right shoulder pain and swelling at the posterior lateral area of the shoulder. As accurate diagnosis is crucial in such case, investigations that included x-rays and magnetic resonance imaging (MRI) demonstrated an soft tissue tumor involving the right shoulder area leading to the differential diagnosis of aggressive soft tissue tumor which laid down the plan of an open incisional biopsy to be reported histopathological as a case of Malignant Triton Tumor which is a very rare and aggressive sarcoma originates from the peripheral nerve sheaths as it is subtype of malignant peripheral nerve sheath tumors after which excision of the entire tumor with safety margin was performed and referred for adjuvant chemotherapy. Conclusion: The treatment of choice is radical tumor excision with wide margins followed by chemotherapy and /or radiotherapy to improve the 5 years survival rates.
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Neurilemoma , Neurofibrosarcoma , Neoplasias Cutáneas , Neoplasias de los Tejidos Blandos , Masculino , Humanos , Persona de Mediana Edad , Neurilemoma/diagnóstico , Neurilemoma/patología , Neurilemoma/cirugía , Neurofibrosarcoma/diagnóstico , Neurofibrosarcoma/cirugía , Hombro/patología , Imagen por Resonancia MagnéticaRESUMEN
Background: Myocarditis refers to myocardial inflammation with necrosis caused by non-infectious of infectious agents such as bacteria, fungi, or drugs. Candida is known to cause myocarditis in healthy and immunocompromised individuals. Diabetes mellitus causes chronic hyperglycemia due to impaired secretion or hypofunction of insulin, induces a compromised state, and increases the risk of contracting various infections. Objective: We report a case of granulomatous myocarditis caused by Candida in a Spontaneously Diabetic Torii rat, a non-obese diabetic model. Case report: A male SDT rat, 61 weeks of age, was housed in conventional environment. The rat was provided a commercial diet and tap water ad libitum. The heart was sampled and prepared the specimen of hematoxylin-and-eosin-, Sirius-red-, Giemsa-, Grocott-stain. Histologically, formation of large granulation tissue was observed in the left ventricular wall. A center of the foci showed necrosis. Moreover, inflammatory cells infiltration and fibrous component were increased surrounding the foci and between myocardial cells. A Grocott and Giemsa staining-positive cell masses occasionally appearing in the foci were considered to be Candida because of their characteristic form. Conclusion: The development and progression of myocarditis were potentially related to a diabetes-induced compromised state.
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Diabetes Mellitus Tipo 2 , Miocarditis , Ratas , Masculino , Animales , Modelos Animales de Enfermedad , Miocarditis/etiología , Necrosis , CandidaRESUMEN
Introduction Malignant triton tumor (MTT) is a rare and aggressive subtype of malignant peripheral nerve sheath tumor consisting of a neurogenic tumor with rhabdomyoblastic differentiation. Only 170 cases have been reported to date, two-thirds occurring in young patients with neurofibromatosis type 1 and the remaining third presenting as a sporadic tumor. Case presentation We present the case of a 49-year-old man with a sporadic grade 2 MTT of the lower limb which had had a previous tibial fracture. The patient underwent an above-knee amputation. Five months post-operatively metastases were present in the liver and vertebral column causing compression of the spinal cord, so decompressive radiotherapy and palliative chemotherapy were initiated. Conclusion Due to the precocious spread of the disease, we would suggest that adjuvant chemotherapy be considered for the eradication of micrometastases. To our knowledge, this is only the second reported case of an MTT arising in a site with a history of previous severe trauma. (AU)
Introducción El tumor tritón maligno (MTT) es un subtipo raro y agresivo de tumor maligno de la vaina del nervio periférico que consiste en un tumor neurogénico con diferenciación rabdomioblástica. Hasta la fecha solo se han descrito 170 casos, dos tercios de ellos en pacientes jóvenes con neurofibromatosis tipo 1 y el tercio restante como tumor esporádico. Presentación del caso Presentamos el caso de un varón de 49 años con un MTT esporádico de grado 2 de la extremidad inferior que había tenido una fractura tibial previa. El paciente fue sometido a una amputación por encima de la rodilla. A los 5 meses del postoperatorio presentaba metástasis en el hígado y en la columna vertebral que causaban compresión de la médula espinal, por lo que se inició radioterapia descompresiva y quimioterapia paliativa. Conclusión Debido a la diseminación precoz de la enfermedad, sugerimos que se considere la quimioterapia adyuvante para la erradicación de las micrometástasis. Hasta donde sabemos, este es solo el segundo caso descrito de un MTT surgido en un lugar con antecedentes de traumatismo grave previo. (AU)
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Humanos , Masculino , Persona de Mediana Edad , Neurofibrosarcoma , Procedimientos OrtopédicosRESUMEN
Introduction Malignant triton tumor (MTT) is a rare and aggressive subtype of malignant peripheral nerve sheath tumor consisting of a neurogenic tumor with rhabdomyoblastic differentiation. Only 170 cases have been reported to date, two-thirds occurring in young patients with neurofibromatosis type 1 and the remaining third presenting as a sporadic tumor. Case presentation We present the case of a 49-year-old man with a sporadic grade 2 MTT of the lower limb which had had a previous tibial fracture. The patient underwent an above-knee amputation. Five months post-operatively metastases were present in the liver and vertebral column causing compression of the spinal cord, so decompressive radiotherapy and palliative chemotherapy were initiated. Conclusion Due to the precocious spread of the disease, we would suggest that adjuvant chemotherapy be considered for the eradication of micrometastases. To our knowledge, this is only the second reported case of an MTT arising in a site with a history of previous severe trauma. (AU)
Introducción El tumor tritón maligno (MTT) es un subtipo raro y agresivo de tumor maligno de la vaina del nervio periférico que consiste en un tumor neurogénico con diferenciación rabdomioblástica. Hasta la fecha solo se han descrito 170 casos, dos tercios de ellos en pacientes jóvenes con neurofibromatosis tipo 1 y el tercio restante como tumor esporádico. Presentación del caso Presentamos el caso de un varón de 49 años con un MTT esporádico de grado 2 de la extremidad inferior que había tenido una fractura tibial previa. El paciente fue sometido a una amputación por encima de la rodilla. A los 5 meses del postoperatorio presentaba metástasis en el hígado y en la columna vertebral que causaban compresión de la médula espinal, por lo que se inició radioterapia descompresiva y quimioterapia paliativa. Conclusión Debido a la diseminación precoz de la enfermedad, sugerimos que se considere la quimioterapia adyuvante para la erradicación de las micrometástasis. Hasta donde sabemos, este es solo el segundo caso descrito de un MTT surgido en un lugar con antecedentes de traumatismo grave previo. (AU)
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Humanos , Masculino , Persona de Mediana Edad , Neurofibrosarcoma , Procedimientos OrtopédicosRESUMEN
Background: Malignant Triton tumor (MTT) is a relatively rare subtype of malignant peripheral nerve sheath tumor (MPNST) characterized by rhabdomyosarcoma differentiation. There are no distinct features of MTT, and it is easy to misdiagnose preoperatively. Case presentation: Here, we describe a rare case of primary hepatic MTT in a 56-year-old male who presented with nonspecific abdominal pain for 1 day. Magnetic resonance imaging and abdominal computed tomography revealed an extremely large mass located in the right liver with intratumoral hemorrhage, arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging and the possibility of intrahepatic metastasis. Tumor marker levels revealed only an elevated level of alpha-fetoprotein (AFP: 5304.0 ng/mL). Then, he received transcatheter arterial chemoembolization combined with lenvatinib and pembrolizumab, and he was diagnosed with hepatocellular carcinoma. After 3 months of neoadjuvant therapy, we resected the hepatic cancer and adherent diaphragmatic pleura. MTT was confirmed by postoperative pathology and immunohistochemistry. Conclusion: Despite the preoperative diagnosis of hepatocellular carcinoma with a rising serum AFP level, typical CT and MRI findings, histopathology assessment showing MPNST with rhabdomyosarcoma differentiation confirms the diagnosis of primary hepatic MTT.
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INTRODUCTION: Malignant triton tumor (MTT) is a rare and aggressive subtype of malignant peripheral nerve sheath tumor consisting of a neurogenic tumor with rhabdomyoblastic differentiation. Only 170 cases have been reported to date, two-thirds occurring in young patients with neurofibromatosis type 1 and the remaining third presenting as a sporadic tumor. CASE PRESENTATION: We present the case of a 49-year-old man with a sporadic grade 2 MTT of the lower limb which had had a previous tibial fracture. The patient underwent an above-knee amputation. Five months post-operatively metastases were present in the liver and vertebral column causing compression of the spinal cord, so decompressive radiotherapy and palliative chemotherapy were initiated. CONCLUSION: Due to the precocious spread of the disease, we would suggest that adjuvant chemotherapy be considered for the eradication of micrometastases. To our knowledge, this is only the second reported case of an MTT arising in a site with a history of previous severe trauma.
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Neurofibrosarcoma , Neoplasias Cutáneas , Masculino , Humanos , Persona de Mediana Edad , Extremidad Inferior , Hígado , Micrometástasis de NeoplasiaRESUMEN
Malignant triton tumor (MTT) is a highly aggressive malignant neoplasm, classified as a variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. There are few reports that MTT occurred in urogenital system. In the present study, we report the first MTT occurring in the uterus. A 57-year-old woman came to the emergency department due to persistent vaginal bleeding for 2 months. The gynecological palpation found that a club-shaped excrescence existed in the vagina about 7 cm × 3 cm × 3 cm. The mass located in the lower segment of the uterus and the cervix was confirmed by gynecological vaginal ultrasound and magnetic resonance imaging, which was preliminarily diagnosed as cervical carcinoma. After neoplasm punch biopsy, the pathological diagnosis was malignant triton tumor. The patient finally lost follow-up. This is the first report about MTT in the uterus and suggests that pathological biopsy combined with imaging examination is necessary for the diagnosis of rarely MTT.
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Neoplasias de la Vaina del Nervio , Neurilemoma , Neurofibrosarcoma , Neoplasias Cutáneas , Femenino , Humanos , Persona de Mediana Edad , Neoplasias de la Vaina del Nervio/patología , Útero/diagnóstico por imagen , Útero/patologíaRESUMEN
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.
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Neoplasias de la Vaina del Nervio , Neurofibromatosis 1 , Neurofibrosarcoma , Humanos , Masculino , Femenino , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Anciano , Neurofibrosarcoma/patología , Neoplasias de la Vaina del Nervio/cirugía , Neoplasias de la Vaina del Nervio/diagnóstico , Estudios Retrospectivos , Resultado del Tratamiento , Neurofibromatosis 1/patologíaRESUMEN
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.
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Malignant Triton Tumors (MTTs) are a rare and aggressive subtype of malignant peripheral nerve sheath tumors (MPNSTs), often associated with neurofibromatosis type 1. This case report describes a unique instance of recurrent sporadic MTT within the carotid sheath in a 33-year-old male without any personal or familial history of neurofibromatosis. The patient initially presented with a biopsy-confirmed MTT in the right neck, involving the carotid body and brachial plexus, and underwent partial resection, radiation therapy, and chemotherapy. Six months later, the patient presented with recurrent MTT, and subsequently underwent radical tumor resection, segmental right carotid artery resection, and deep femoral vein interposition. Recovery was complicated by hematoma formation, and the patient developed vocal fold paralysis and a left vocal fold cyst, necessitating further surgeries. Yearly follow-ups for 8 years revealed no recurrence. This case emphasizes the importance of comprehensive patient evaluation, including clinical history, imaging, and biopsy findings, for accurate diagnosis and prompt surgical intervention in managing such rare and aggressive tumors. Further research is needed to identify novel therapies and improve survival rates for patients with MTTs.
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PURPOSE: Benign triton tumors (BTTs) in the pediatric population are extremely rare occurrences. Paucity of data on BTTs poses both diagnostic and therapeutic challenges, particularly when found intracranially. METHODS: A case report of a 10-year-old male diagnosed with incidental maxillary trigeminal (V2) BTT is presented. We discuss radiographic and histopathological interpretations. Furthermore, we provide a brief review of current literature and historical background on pediatric trigeminal BTT diagnosis, histopathology, and management. RESULTS: Successful gross total resection of the tumor was achieved via Dolenc approach to the cavernous sinus. Management options with consideration of outcomes from the few prior cases reported in the literature are presented. CONCLUSION: Treatment of trigeminal nerve tumors requires a broad differential diagnosis and understanding rare tumors is essential in the diagnosis and treatment algorithm.
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Seno Cavernoso , Neoplasias de los Nervios Craneales , Hamartoma , Enfermedades del Nervio Trigémino , Masculino , Niño , Humanos , Nervio Trigémino/diagnóstico por imagen , Nervio Trigémino/cirugía , Nervio Trigémino/patología , Neoplasias de los Nervios Craneales/diagnóstico por imagen , Neoplasias de los Nervios Craneales/cirugía , Enfermedades del Nervio Trigémino/diagnóstico por imagen , Enfermedades del Nervio Trigémino/cirugía , Hamartoma/patología , Seno Cavernoso/cirugíaRESUMEN
BACKGROUND: Malignant 'triton' tumor is an extremely rare subtype of malignant periphery nerve sheath tumors. Clinical diagnosis of malignant triton tumor is difficult before surgery due to its low incidence and the lack of knowledge. Therefore, to describe and summarize the CT imaging characteristics of malignant triton tumor is of great assistance for early and preoperative diagnosis. CASE PRESENTATION: Two cases suspected of MTT by CT scan before operation were closely observed. The diagnosis of malignant triton tumor was eventually confirmed by immunochemical assay, which verified speculation of CT scans. Huge, irregular, well-circumscribed lobulated mass-like shadows can be observed from these patients by CT scans. Besides, heterogeneity of density within the body of tumor was well-established by CT scans, together with linear septum. Meanwhile, CT scans demonstrated that calcifications were remarkable at the margin of tumor body. CONCLUSIONS: Some CT image features from two cases were presented as a reference for the preoperative consideration of MTT: (i) enormity of mass-like shadow; (ii) presence of well-circumscribed lobulated shape; (iii) septum within the well-defined mass accompanied with hemorrhage, necrosis and cystic changes as well as calcification, especially within neurofibromatosis type 1 patients.
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Neoplasias Óseas , Neoplasias de la Mama , Femenino , Humanos , Tomografía Computarizada por Rayos XRESUMEN
This is an additional case report of a malignant triton tumor arising in the duodenum that was removed by pancreatoduodenectomy. Liver and gallbladder dysfunctions were detected in a regular blood examination during a follow-up for hypertension in a 62-year-old woman with a previous surgical history for a malignant Triton tumor in the duodenum 13 years ago. Further examinations revealed a metastatic liver tumor originating from the malignant triton tumor in the duodenum. Since the progression of the liver tumor was detected after radiation therapy, complete resection was performed by right hepatectomy. Curative hepatectomy resulted in disease-free survival for 1 year and 5 months in an extremely rare case of liver metastasis derived from a malignant triton tumor in the duodenum.
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BACKGROUND: Neuromuscular choristoma (NMC) is a rare peripheral nerve lesion that is composed of ectopic mature muscle fibers and nerve fascicles, typically involving major nerve roots or trunks, such as the cranial nerves, brachial plexus, and sciatic nerves. The onset of NMC frequently occurs in the first decade of life. Here, we present the first documented case of a case of esophageal NMC in an adult patient. CASE PRESENTATION: A 46-year-old male patient presented in 2018 with a submucosal tumor of the esophagus. Upon presentation, the tumor was approximately 10 mm in diameter, covered by normal mucosa, and located in the left posterior wall of the esophagus in a position that was 30 cm from the incisor. The tumor was discovered incidentally during gastroscopic examination. In March 2021, endoscopic re-examination revealed no significant changes in the tumor. Endoscopic ultrasound revealed an oval hypoechoic mass with a homogeneous internal echo that originated from the muscularis propria with a maximum cross section of 13 mm × 6 mm. Resection was performed under gastroscopy. The resection specimen was 12 mm × 5 mm in size and was a well-demarcated, elastic, hard, and tough with a gray section. Histologically, the specimen consisted of an abundance of smooth muscle fiber bundles intercalated among nerve fibers, but without malignancy. Immunohistochemical examinations revealed positivity for S-100 protein, caldesmon, NSE and desmin, but negativity for CD117, DOG-1, HMB45, and Melan A. There was also aberrant nuclear localization of beta-catenin. Collectively, these findings led to a diagnosis of esophageal NMC. CONCLUSIONS: NMC is extremely rare, especially esophageal NMC, and is very challenging to accurately diagnose prior to resection. It is important that we can differentiate NMC from other types of tumors.
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Coristoma , Neoplasias Esofágicas , Hamartoma , Coristoma/diagnóstico por imagen , Coristoma/patología , Coristoma/cirugía , Endosonografía , Neoplasias Esofágicas/diagnóstico , Neoplasias Esofágicas/patología , Neoplasias Esofágicas/cirugía , Esófago/patología , Humanos , Masculino , Persona de Mediana Edad , Músculo Esquelético/patología , Proteínas S100RESUMEN
Malignant Triton tumor (MTT) is a rare variant of malignant peripheral nerve sheath tumor which harbingers a poor prognosis owing to its aggressive behavior. We report a case of a gentleman, who presented with MTT of the lower alveolus, and management with extensive extirpative surgery and adjuvant radiotherapy.
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Introducción: El tumor tritón maligno es una neoplasia rara en la que se encuentran células rabdomioblásticas en un tumor maligno de la vaina de nervios periféricos, que se caracteriza por su agresividad y mal pronóstico. La localización en la cabeza y el cuello es poco frecuente. La inmunohistoquímica juega un papel importante en el diagnóstico. Objetivo: Describir un tumor tritón maligno de tamaño inusual. Presentación del caso: Paciente femenino, de 16 años, es referida al servicio de cirugía maxilofacial del Instituto Nacional de Pediatría, Ciudad de México, con un diagnóstico de tumor neuroectodérmico en región facial y cervical de un año de evolución. Clínicamente el tumor era exofítico, multilobulado, con zonas extensas de necrosis, superficie de varias tonalidades y un tamaño aproximado de 18 x 10 x 12 cm. Se realizó una biopsia e inmunohistoquímica que confirmó el diagnóstico de tumor tritón maligno. La paciente fue intervenida quirúrgicamente, procedimiento con el cual se eliminó totalmente la lesión, con márgenes de seguridad. La paciente presentó una evolución tórpida, con desenlace fatal al cabo de seis meses del tratamiento. Conclusiones: El tumor tritón es una neoplasia agresiva y su detección oportuna orienta al cirujano a ofrecer al paciente un tratamiento adecuado(AU)
Introduction: Malignant triton tumor is a rare neoplasm in which rhabdomyoblasts are present in a malignant tumor of the peripheral nerve sheath. This condition is characterized by its aggressiveness and bad prognosis. Location in the head and neck is infrequent. Immunohistochemical testing plays an important role in its diagnosis. Objective: Describe an unusually large malignant triton tumor. Case presentation: A case is presented of a female 16-year-old patient referred to the maxillofacial surgery service of the National Institute of Pediatrics in Mexico City with a diagnosis of neuroectodermal tumor of one year's evolution in the facial and cervical region. In clinical terms, the tumor was exophytic, multilobed, with extensive areas of necrosis, a surface in several shades of color and an approximate size of 18 x 10 x 12 cm. Biopsy and immunohistochemical testing confirmed the diagnosis of malignant triton tumor. The patient underwent surgery in which the lesion was totally excised with a safety margin. Evolution was clumsy, with a fatal outcome at six months of treatment. Conclusions: Triton tumor is an aggressive neoplasm whose early detection makes it possible for surgeons to provide an appropriate treatment(AU)
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Humanos , Cirugía Bucal , Biopsia , Tumores Neuroectodérmicos/etiología , Resultado Fatal , Selección del Sitio de Tratamiento de ResiduosRESUMEN
Under extreme conditions and in palliative settings, shared decision making with the patient is vital; narrative decisions beyond evidence could be considered. If there is a chance of symptom palliation, extended surgery should not be avoided merely because of the limited life expectancy.
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Malignant peripheral nerve sheath tumor (MPNST) is a very aggressive peripheral nerve sheath-derived sarcoma, which is one of the most difficult tumors to diagnose due to its wide spectrum of histological findings and lack of specific immunohistochemical markers. Recently, it has been reported that losses of expression of H3K27me3 and H3K27me2 caused by PRC2 dysfunction may be useful diagnostic markers for MPNST, but there is no consensus on their clinicopathological significance. Here, we investigated the relationship between loss of H3K27 methylation and various parameters and clarified the clinicopathological significance of such loss. We analyzed the clinicopathological and immunohistochemical features in 84 MPNST cases. Complete losses of H3K27me3 and H3K27me2 were observed in 37 (44%) and 29 (35%) cases, respectively. Losses of H3K27me3 and H3K27me2 were significantly correlated with myogenic immunopositivity (H3K27me3 vs. desmin, P = 0.0051; H3K27me3 vs. myogenin, P = 0.0009; H3K27me2 vs. myogenin, P = 0.042). Meanwhile, there were significant correlations between preservation of immunohistochemical neurogenic markers and intact H3K27me3 and H3K27me2 (H3K27me3 vs. S-100 protein, P = 0.0019; H3K27me3 vs. SOX10, P = 0.014; H3K27me2 vs. S-100 protein, P = 0.0011; H3K27me2 vs. SOX10, P = 0.0087). In multivariate analysis, local recurrence, distant metastasis, high FNCLCC grade, and loss of SOX10 expression were independent prognostic factors for overall survival. H3K27me3 and H3K27me2 expression was retained in all 26 cases of rhabdomyosarcoma non-alveolar subtype. In conclusion, we suggest that H3K27me3 and H3K27me2 immunonegativity is useful but not definitive for diagnosing MPNST. Complete loss of H3K27 methylation may be involved in aggressive transdifferentiation from neural differentiation to skeletal muscle differentiation in MPNST.