Intracranial metastasis from a malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1: A case study and literature review.

INTRODUCTION: Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are a rare type of soft tissue sarcoma. While these tumors often metastasize, intracranial metastases of MPNSTs have only been rarely noted. METHODS: Using Pubmed, Google Scholar, and Science Direct we conducted a systematic review of the literature to identify all reported cases of MPNSTs with metastases to the brain since the inception of these databases through January 2020. Data were extracted and data analysis was completed using python statistical packages. RESULTS: Only 26 cases (including present study) of MPNSTs resulting in intracranial metastases have been reported in the literature. Eight of these 26 cases occurred in patients who were previously diagnosed with Neurofibromatosis Type 1 (NF1). Additionally, one patient had been previously diagnosed with Neurofibromatosis Type 2 (NF2). The average reported time from diagnosis of a MPNST to the time of diagnosis with intracranial metastasis was 36 months, with a median time of 14 months. The average reported survival time for patients after being diagnosed with intracranial metastasis was 5.9 months. The cases that utilized a combination of therapeutic intervention including surgical resection, radiotherapy and chemotherapy saw the greatest improvement of survival times. CONCLUSION: MPNSTs with brain metastases are extremely rare and have a poor prognosis with a 6 months median survival after metastasis. While combination therapy is indicated, further studies on treatment are needed to determine survival benefits. Early and effective initial diagnosis of MPNST before brain metastases occurs is likely to give the best chance of increased overall survival.

Incidence and prognosis of distant metastasis in malignant peripheral nerve sheath tumors.

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are a type of soft tissue sarcomas (STS) with recurrence and metastatic potential. We aimed to investigate the risk factors for developing distant metastases (DM) and to identify the prognostic factors in patients with DM. METHODS: Based on the Surveillance, Epidemiology, and End Result (SEER) database, MPNST patients diagnosed between 2010 and 2016 were extracted in our study. The logistic regression model was performed for predicting DM development while the Cox proportional hazard regression model was conducted for revealing the prognostic factors. RESULTS: Eventually, 764 patients diagnosed with MPNSTs were included with 109 cases presenting with metastases at initial diagnosis. Larger tumor size and lymph node metastases were independent risk factors for developing DM. The median overall survival (OS) for patients with metastases was 8.0 (95% CI: 6.1-9.9) months. Multiple metastatic sites and no surgical treatment were prognostic factors for worse survival. Tumors located in non-head and neck region were related with better survival. CONCLUSIONS: The incidence of DM was 14.3% with a dismal median OS of 8.0 months for metastatic MPNSTs. More evaluation should be applied for patients with large tumor size and lymph metastases. Tumors located in head and neck region and the presence of multiple metastases predicted worse survival outcome. Surgical treatment can significantly improve the survival of MPNST patients with distant metastasis.

[Intracerebrally localized sarcoma NOS--an interdisciplinary challenge]. / Intrazerebral lokalisiertes Sarkom NOS--eine interdisziplinäre Herausforderung.

BACKGROUND: In the literature, surveys of malignant intracerebral nerve sheath tumors are very rare (Table 1). Therapeutic guidelines do not exist. CASE REPORT: A 28-year-old female patient presented with a tumor in the postcentral region of the left parietal lobe (Figures 1 and 2). The specimen could not be categorized into a common tumor entity and was classified as sarcoma NOS. Shortly after surgery, a recurrence occurred (Figures 3 to 5) followed by a further excision. Due to the rapid tumor growth irradiation with CT-aided treatment planning (Figure 6) has been started immediately afterwards. A dose of 5,400 cGy in 22 fractions was administered. RESULT: 2 weeks after treatment, the patient presented with a noticeable tumor regression (magnetic resonance imaging; Figures 7 to 9). She developed pulmonary metastases. A partial remission could be achieved by systemic chemotherapy. Unfortunately, the patient died because of an exacerbation of a hepatic encephalopathy. CONCLUSION: In cases of intracerebrally localized sarcomas NOS, the earliest possible start of radiotherapy after surgery seems useful because of the noticed radiosensitivity of these tumors. In regard of the local control, this tumor entity shows a documented excellent response to radiotherapy. Expectedly, distant metastases cannot be influenced. Interdisciplinary cooperation is mandatory to enhance the diagnostic process, the treatment decisions, and the results.

Primary and secondary heart tumours in dogs and cats.

Primary and secondary neoplasms of the canine and feline heart are uncommon. During a 2-year period, 83 dogs suffering from primary cardiac (n=11), extracardiac benign (n=6) or malignant (n=66) tumours and 30 cats with primary cardiac (n=1) or extracardiac (n=29) malignant tumours were examined. Echocardiography revealed four cases of primary cardiac neoplasms in dogs, but secondary heart tumours were not detected. After necropsy, tissue samples from the heart and tumours were examined histologically and immunohistochemically. In dogs, primary neoplasms included seven haemangiosarcomas, two chemodectomas, one rhabdomyosarcoma, and one neurofibrosarcoma. In 24 of 66 dogs examined, metastases of extracardiac neoplasms were found in the heart (15 carcinomas, six malignant lymphomas, three haemangiosarcomas). In cats, one case of primary haemangiosarcoma of the pericardium and five cases of secondary cardiac tumours (two malignant lymphomas, three carcinomas) occurred. Cardiac neoplasms in cats were not identified clinically but were detected by detailed gross sectioning of the heart (n=2) or histopathological examinations (n=3). This study showed an unexpectedly high number (36%) of dogs with cardiac metastases.

Pediatric neurofibrosarcoma.

BACKGROUND/PURPOSE: Neurofibrosarcoma is rare in children, and the natural history and prognostic factors are not well described. The authors present a 57-year review of their experience. METHODS: The charts of children with neurofibrosarcoma were reviewed retrospectively. Statistical analysis was performed using the Chi2 and unpaired t tests. RESULTS: From 1944 to 2001, 38 patients under the age of 21 were diagnosed with neurofibrosarcoma. Twenty-two were boys. The average age at diagnosis was 13.8 years (range, 3 to 19.9 years). Nineteen patients (50%) had neurofibromatosis. The tumor site was as follows: extremity, 19 patients; trunk, 9 patients; head and neck, 7 patients; and retroperitoneum, 3 patients. The average tumor size was 10 cm. The margins after resection were as follows: grossly positive, 9; microscopically positive, 5; negative, 21; and unknown, 3. Patients with positive margins had a 22% survival rate, whereas those with negative or unknown margins had a 34% survival rate. Thirty-two patients achieved a complete response, 2 a partial response, and 4 progressed while on therapy. Twenty-six patients relapsed after a complete response (11 local, 10 distant, 5 both). Of the 15 patients with a distant relapse, 73% (11) relapsed in the lung. Twelve (32%) patients survived with an average follow-up of 14 years (range, 0.3 to 28 years). Nine patients were treated with chemotherapy, 9 with radiation, and 9 with both chemotherapy and radiation. Outcome was not significantly affected by gender, presence of neurofibromatosis, site, margin, or use of adjuvant therapy. CONCLUSION: Neurofibrosarcoma remains a rare disease in children with insufficient contemporary numbers to assess efficacy of therapy. Prognosis remains poor with a high incidence of relapse, particularly in the lungs, suggesting that more aggressive therapies to control both local and distant relapses are needed.

Sciatic nerve resection: is that truly an indication for amputation?

En bloc resection of the sciatic nerve with an adjacent bone or soft tissue tumor has been assumed to be associated with a poor functional outcome and, therefore, was considered an indication for amputation. Although many surgical oncologists today challenge this assumption and do limb-sparing resection in these patients, a report of the functional outcome of a series of patients who had this procedure has not been published. Between 1991 and 1999, the authors treated 15 patients who had resection of the sciatic nerve. There were 10 females and five males, ranging in age from 2 to 73 years. Diagnoses included 11 high-grade soft tissue sarcomas, one primary bone sarcoma, and three metastatic bone tumors. Four lesions were located in the pelvis, one in the buttock, and 10 in the posterior thigh. At the most recent followup, 14 patients were ambulatory, seven of whom required a walking aid. Because of peroneal nerve palsy, all patients required a short-leg brace. However, overall function was determined to be good in 11, moderate in three, and poor in one patient. None had a pressure sore of the foot and none required a secondary amputation. Good function is achieved in most patients who have sciatic nerve resection. Therefore, the necessity to resect the sciatic nerve is not an indication for amputation.

Metastatic spinal neurofibrosarcoma.

Neurofibrosarcomas are rare tumors usually arising in somatic soft tissues or peripheral nerves. Four cases of metastatic neurofibrosarcoma to the spine have been reported before. The current case is unusual because of the presence of two distinct, metachronous spinal metastasis and lung metastasis. A 30-year-old woman with neurofibromatosis and a history of previous neurofibrosarcoma resection presented with back pain. Radiologic evaluation revealed a lytic lesion of the eleventh thoracic vertebra. A transthoracal corpectomy, reconstruction by Harms' cage and posterior instrumentation, and fusion were carried out. After the completion of adjuvant chemotherapy, a solitary pulmonary nodule was detected. Shortly after resection of the metastatic pulmonary nodule, the patient complained of acute onset low-back pain. Radiologic assessment revealed another lytic lesion in the L5 vertebra after 6 months. Again, a corpectomy, anterior and posterior instrumentation, and fusion were carried out. Eight months after the second spinal resection, another solitary pulmonary metastasis was diagnosed and resected. The patient's health status suddenly deteriorated 26 months after the initial spinal metastatectomy, and she died. Though local control can be achieved in more than 80% of the patients with neurofibrosarcoma by wide surgical resection followed by adjuvant chemo- and radiotherapy, most patients die of systemic metastasis. The current patient survived 50 months after the initial resection of a forearm neurofibrosarcoma. Despite achieving local control, she died due to systemic recurrence. Prolonged survival with the help of chemo- and radiotherapy justifies our aggressive surgical strategy for the treatment of spinal metastasis in order to achieve neurologic cure and spinal stability.

Malignant spinal neurofibrosarcoma.

STUDY DESIGN: A report of a case of metastatic spinal neurofibrosarcoma. OBJECTIVE: To document metastatic neurofibrosarcoma as a cause of spinal cord compression and to review the literature. SUMMARY OF BACKGROUND DATA: Three previously reported cases of metastatic neurofibrosarcoma of the spine were reviewed. METHODS: The patient's clinical record and radiologic investigations as well as the result of a search of the English literature are reported. Magnetic resonance images, computed tomographic scans, and histology photomicrographs are displayed. RESULTS: Paraparesis developed in this patient, due to a posterior extradural thoracic spinal cord compression by a neurofibrosarcoma believed to be metastatic from a neurofibrosarcoma of the femoral nerve. CONCLUSIONS: Malignant spinal metastasis remains a rare complication of neurofibromatosis, with a very poor prognosis.

[A case of maxillary neurosarcoma]. / Przypadek nerwiakomiesaka (neurosarcoma) szczeki.

A case of neurosarcoma located in maxillary sinus is presented. The patient was 62 years old women who complained of nasal obstruction, epistaxis and headache. Results of physical examination showed a giant mass filling the nasal cavity and maxillary sinus. The lesion was completely resected by lateral rhinotomy way. After the operation histopathological diagnosis was difficult, one pathologist recognised schwannoma benignum other one sarcoma. Finally postoperative diagnosis was schwannoma benignum, it was misdiagnosis. Two years later the patient was surgically treated again because of tumor recurrence. These time pathological diagnosis was neurosarcoma. Radiotherapy was done as well. The patient died 18 months after treatment, tumor regrowth and generalization metastases was reason of the death.

[Neurosarcoma of the submandibular gland arising in a pleomorphic adenoma. Apropos of a case]. / Neurosarcome de la glande submandibulaire développé sur un adénome pléomorphe. A propos d'un cas.

A 71-year-old woman consulted for a tumor of the right submandibular gland that had been present for 4 years and had rapidly increased in size during the last two months. Histological examination of frozen sections and immunohistochemistry concluded to a poor differentiated sarcoma probably a neurosarcoma with residual pleomorphic adenoma component. In spite of wide resection, local recurrences with nodes and lung metastases occurred leading to the death of the patient. We think interesting to report this unusual salivary tumor and to discuss its pathological features.

[Malignant tumors of the peripheral nerve sheath (MPNST) after irradiation]. / Tumore maligno della guaina dei nervi periferici (MPNST) post-irradiazione.

A case of relapsed malignant tumor of the brachial plexus and its nervous roots (MPNST) was observed in a patient treated with radiotherapy for Hodgkin's disease 24 years previously. A known risk of radiation therapy is the induction of secondary neoplasms, less commonly malignant peripheral nerve sheath tumor (MPNST). The clinico-pathological features of this rare tumor are presented and the possible relationship between radiotherapy and MPNST is discussed.

[Neurosarcoma associated with neurofibromatosis 1. Apropos of a case and review of the literature]. / Neurosarcome associé à une neurofibromatose de type 1. A propos d'un cas et revue de la littérature.

BACKGROUND: Type 1 neurofibromatosis considerably increases the risk of cancer development, particularly neurosarcoma. We report a case in a patient with chemosensitive metastatic neurosarcoma. CASE REPORT: A young female patient with familial type 1 neurofibromatosis developed pleural metastasis of a neurosarcoma located on the arm. This tumor was initially highly sensitive to chemotherapy, but relapse occurred. DISCUSSION: Follow-up in the order members of the family was particularly difficult to organize. One sister developed cerebral astrocytoma. Neurosarcomas develop earlier in patients with type 1 neurofibromatosis, worsening prognosis. We suggest a prospective and structured registration of such cases using a network of clinicians and pathologists in order to improve management schemes.