Transoral Robotic Surgery in the Management of a Tongue Base Schwannoma.

Schwannomas are the most common benign tumors arising from the peripheral nerve sheath, and the intraoral location is very atypical, representing less than 1% of all cases. Surgical excision is the treatment of choice, and a variety of surgical approaches have been described. The authors report the first described case of tongue base schwannoma treated with transoral robotic surgery (TORS). A 47-years-old female patient complaining mild dysphagia and snoring, presented a submucosal swelling at the right side of the tongue base. MRI showed a large well-circumscribed solid mass, homogeneously isointense in T1WI and hyperintense on T2WI, with no lymph node metastasis. According to size, location and radiological characteristic of the mass a TORS approach was chosen. An extracapsular dissection was performed, and the lesion was completely removed with no intraoperative complications. The final diagnosis based on histopathological examination and IHC analysis (S-100 positive) was a schwannoma of the tongue base. The post-operative course was uneventful, and no recurrence was observed after 6 months of follow-up. This study demonstrates the feasibility of TORS in the treatment of a tongue base schwannoma. This is a valid alternative to the common transoral approach in order to avoid more invasive external approaches, and further studies are recommended in order to clarify if this approach could be proposed as the first line treatment in selected cases.

Natural Orifice-Assisted Laparoscopic Meckel Diverticulectomy Incidentally Found During Para-Aortic Mass Resection.

STUDY OBJECTIVE: To show a surgical video in which an incidentally found Meckel diverticulum was resected with a natural orifice-assisted laparoscopic approach during para-aortic resection of a retroperitoneal schwannoma. DESIGN: Case report (Canadian Task Force classification III). SETTING: Tertiary referral center in New Haven, Connecticut. INTERVENTIONS: This is a step-by-step illustration for resection of a retroperitoneal para-aortic schwannoma and of an incidentally found Meckel diverticulum. The patient was a 39-year-old white woman diagnosed with stage IV choriocarcinoma with metastasis to the lungs and left para-aortic area. She received chemotherapy in the form of etoposide, methotrexate, actinomycin-D, cyclophosphamide, oncovine (EMA-CO) and had an excellent clinical response with resolution of all metastatic disease except for the para-aortic mass. Therefore, she was taken to the operating room for laparoscopic resection of the persistent left para-aortic mass. After placement of four 5-mm abdominal ports, the pelvis and abdomen were explored and revealed an incidental Meckel diverticulum as well as the 5 cm left para-aortic mass. The peritoneum overlying the para-aortic mass was incised and the retroperitoneum explored. Given the proximity to the mass, left ureterolysis was performed. The retroperitoneal attachments were resected, and the left para-aortic mass was removed without any complications. At this point attention was turned to the Meckel diverticulum. In order not to extend the abdominal incisions, a posterior colpotomy was performed in the cul-de-sac equidistant from the uterosacral ligaments. Endo-GIA (Covidien, New Haven CT) was introduced through the 10-mm port site at the posterior colpotomy. Meckel diverticulum was resected without narrowing the lumen of the distal ileum. The specimen was removed in a contained manner through posterior colpotomy. MEASUREMENTS AND MAIN RESULTS: The procedure was performed without any complications. The patient had an uneventful postoperative course and was discharged home on postoperative day 0. Pathology revealed a retroperitoneal schwannoma with negative margins and benign Meckel diverticulum without ectopic gastric or pancreatic tissue. The patient has been disease-free since the completion of surgery. CONCLUSION: Laparoscopic resection of the retroperitoneal schwannoma and Meckel diverticulum were successfully performed in this patient with history of stage IV choriocarcinoma. To our knowledge, this is the first report describing a natural orifice-assisted laparoscopic approach for resection of Meckel diverticulum. Natural orifice-assisted laparoscopy should be considered when the surgeon needs to remove a large specimen and/or to introduce >5-mm diameter instruments into the peritoneal cavity without having to extend the abdominal incisions.

Metastatic malignant peripheral nerve sheath tumour presenting as a spontaneous extradural haematoma - A case report.

Malignant peripheral nerve sheath tumours may arise from any cranial or somatic nerve. The median survival with best therapy is 49 months. The present case reports a patient with an MPNST that exhibited an unusually indolent behaviour. Besides this, the patient developed a dural metastasis from the lesion and presented with a spontaneous extra-dural haematoma. This has not been reported hitherto in literature.

[Schwannoma in the Middle Mediastinum Originating from the Vagal Nerve；Report of a Case].

A 44-year-old woman was referred to our department with a mediastinal tumor detected by computed tomography performed as a preoperative examination for cervical cancer. There was a 32 mm solid mass in the area surrounded by the descending thoracic aorta, esophagus, left atrium, left lower lobe, and mediastinal pleura. The tumor was removed thoracoscopically. The mass was regarded as a neurogenic tumor arisen from the branch of the vagus nerve. Neither symptoms of postoperative neurological disorders nor signs of recurrence have been noted to date. The histopathological diagnosis was schwannoma.

Tumor-to-tumor metastasis of breast carcinoma to cervical spinal schwannoma: Case report.

Tumor-to-tumor metastasis with schwannoma as a recipient tumor is very rare. There have been no reports on tumor to spinal schwannoma metastasis. We report a case of a 57-year-old woman who presented with neck pain and who turned out to have cervical spinal nerve schwannoma with breast carcinoma metastasis.

[Rare solid pancreatic tumors]. / Rzadko wystepujace lite nowotwory trzustki.

The most common tumor of the pancreas is cancer, which constitutes 85% of all pancreatic neoplasms. Cystic pancreatic tumors comprise 10% of malignancies. No more than 5% of pancreatic tumors are rare solid tumors as: neuroendocrine tumors, gastrointestinal stromal tumors, solid pseudopapillary tumors, pecomas, lymphomas, granulocytic sarcomas, schwannomas, lipomas, liposarcomas and metastases to pancreas. Nowadays, these tumors are diagnosed more commonly due to the developement and accessibility of the diagnostic imaging techniques. Moreover, the treatment and management of rare solid pancreatic tumors often differs from the management in pancreatic cancer what makes the differential diagnosis difficult and responsible challenge. The main purpose of this article is to present an actual data of epidemiology, clinical presentation, management and treatment of rare solid pancreatic tumors according to recent literature and self experience.

Diffusion tensor imaging in spinal cord compression.

BACKGROUND: Although diffusion tensor imaging has been successfully applied in brain research for decades, several main difficulties have hindered its extended utilization in spinal cord imaging. PURPOSE: To assess the feasibility and clinical value of diffusion tensor imaging and tractography for evaluating chronic spinal cord compression. MATERIAL AND METHODS: Single-shot spin-echo echo-planar DT sequences were scanned in 42 spinal cord compression patients and 49 healthy volunteers. The mean values of the apparent diffusion coefficient and fractional anisotropy were measured in region of interest at the cervical and lower thoracic spinal cord. The patients were divided into two groups according to the high signal on T2WI (the SCC-HI group and the SCC-nHI group for with or without high signal). A one-way ANOVA was used. Diffusion tensor tractography was used to visualize the morphological features of normal and impaired white matter. RESULTS: There were no statistically significant differences in the apparent diffusion coefficient and fractional anisotropy values between the different spinal cord segments of the normal subjects. All of the patients in the SCC-HI group had increased apparent diffusion coefficient values and decreased fractional anisotropy values at the lesion level compared to the normal controls. However, there were no statistically significant diffusion index differences between the SCC-nHI group and the normal controls. In the diffusion tensor imaging maps, the normal spinal cord sections were depicted as fiber tracts that were color-encoded to a cephalocaudal orientation. The diffusion tensor images were compressed to different degrees in all of the patients. CONCLUSION: Diffusion tensor imaging and tractography are promising methods for visualizing spinal cord tracts and can provide additional information in clinical studies in spinal cord compresion.

What About Gastric Schwannoma? A Review Article.

PURPOSE: Gastric schwannomas (GSs) are rare mesenchymal neoplasms of the gastrointestinal tract. Diagnosis is often achieved postoperatively, based on pathology reports of retrieved specimens. The aim of the present study is to follow up all patients with gastric schwannoma (Gs) undergoing endoscopic, partial, or more extended surgery and to evaluate the appearance of local or distant recurrence. METHODS: A PubMed, Cochrane, and Embase systematic review of the literature has been performed. Original papers, review articles, and case reports published between 1988 and 2019 were considered eligible. All the studies who met the inclusion criteria were analyzed. Statistical analysis of data has been performed using GraphPad Prism 7 software. RESULTS: Three hundred twenty-eight articles were found, and a total of 102 were included and analyzed in depth. Fifty-three papers reported the follow-up information, ranging from 1 to 417 months across different studies. Among them, 31 patients underwent endoscopic removal of the gastric lesions; 140 patients underwent local surgery, including wedge resection or partial gastrectomy; and 148 patients underwent subtotal or total gastrectomy. The median follow-up was of 27-38-33 months, respectively. No recurrence or distant metastasis was detected in the endoscopy group. Among local surgery group, liver metastasis was reported in one case; in extended surgery group, one patient died for multiple liver metastases. CONCLUSIONS: Local or more extended surgery involved a larger cohort of patients and reported satisfactory long-term results compared with endoscopy group. Surgery in absence of a definite preoperative diagnosis is considered the gold standard treatment for resectable Gs.

Malignant schwannoma in pregnancy: a case report and literature review.

Malignant peripheral nerve sheath tumors in pregnancy are an uncommon finding. This diagnosis in a pregnant patient with neurofibromatosis type 1 poses additional treatment dilemmas. A 28-year-old primigravida with known neurofibromatosis type 1 complained of increasing shortness of breath and cough. Evaluation revealed a large chest mass, biopsy proven to be a malignant schwannoma. This malignant peripheral nerve sheath tumor, discovered at 26 weeks' gestation, grew so rapidly that delivery was necessary at 30 weeks' gestation. Management of pregnant patients with rare, rapidly growing tumors may require early delivery in cases where maternal health is in jeopardy.

Targeted next-generation sequencing of malignant peripheral nerve sheath tumor of the pterygopalatine fossa with intracranial metastatic recurrence.

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon neoplasm that rarely involves the head and neck region. Intracranial MPNSTs unrelated to cranial nerves are highly malignant tumors with poor overall survival, probably because of infiltrating growth into surrounding brain tissue. The pathogenesis of MPNST remains unclear. There are no conclusive explanations for the mechanisms underlying the initiation, progression, and metastasis of MPNST. In this paper, we describe a case of MPNST in the pterygopalatine fossa with intracranial metastatic recurrence and review related literatures. Meanwhile, targeted next-generation sequencing (NGS) revealed the presence of both a beta-catenin (CTNNB1) missense mutation p.Ser33Phe and a mediator complex subunit 12 (MED12) frameshift mutation p.Tyr1278fs in the recurrent intracranial tumor. Therapies that target CTNNB1 mutation, MED12 mutation, CTNNB1 activation, or Wnt pathway activation are worth future studying.

Malignant Peripheral Nerve Sheath Tumor Arising in Schwannomatosis with Multiple Lung Metastases.

BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is a kind of rare neurogenic malignancy, which usually arises from nerve fibers in any tissue and organ that have nerve fiber distributions, especially the trunk and extremities, but it is extremely rare in spinal canal. CASE DESCRIPTION: We report a 30-year-old woman who had a history of excision of intraspinal occupying lesions 5 times and the pathologic diagnosis based on histomorphologic and immunohistochemistry was schwannomatosis, which existed in her family history. Unfortunately, she died because her condition deteriorated rapidly and appeared multiple lung metastases. MPNST was confirmed by needle biopsy of lung lesions. CONCLUSIONS: Many cases of MPNST usually developed from neurofibromatosis type 1. However, the incidence of MPNST arising from schwannomatosis was extremely rare. More significantly, using genetic testing on her, we found a splice site mutation (c.1118+1G>A) that occurred between exons 8 and 9 of the SMARCB1 gene, which was first found in this MPNST patient and could lay the foundation for further study of its pathogenesis.

Laparoscopic partial splenectomy.

BACKGROUND: The immunologic function of the spleen and its important role in immune defense has led to splenic-preserving surgery. This study aimed to evaluate whether laparoscopic partial splenectomy is safe. METHODS: Data on consecutive patients presenting with localized benign or malignant disease of the spleen were included in a prospective database. The surgical technique consisted of six steps: patient positioning and trocar placement, mobilization of the spleen, vascular dissection, parenchymal resection, sealing/tamponading of the transected edge, and removal of the specimen. RESULTS: From 1994 to 2005, 38 patients underwent laparoscopic partial splenectomy. The indications included splenomegaly of unknown origin, splenic cysts, benign tumors (hamartoma), and metastasis from ovarian carcinoma and schwannoma. The median operating time was 110 min (range, 65-148 min). The median length of hospital stay was 5 days (range, 4-7 days). There was no postoperative mortality. Postoperative pleural effusion occurred in two patients. There were no reoperations. Three patients required blood transfusions. CONCLUSION: Laparoscopic partial splenectomy is safe for patients with localized benign or malignant disease of the spleen.

Fine-needle aspiration cytologic diagnosis of metastatic melanotic schwannoma: familial case of a mother and daughter with Carney's complex and literature review.

Carney's complex is an autosomal dominant, multisystem tumorous disorder that includes myxomas, spotty skin pigmentation, endocrine tumors, and peripheral nerve tumors. Psammomatous melanotic schwannomas have recently been included as a part of this complex. Here, we describe the first known familial case of a mother and daughter, both presenting with malignant, already metastatic, pigmented schwannomas initially diagnosed as metastatic melanoma by CT guided fine-needle aspiration. Patients with highly pigmented, extra-cutaneous lesions that are clinically and pathologically suspicious for metastatic malignant melanoma, without known primary tumor, should be evaluated for possible Carney's complex. Additional screening of family members should be recommended to exclude the presence of potentially malignant neoplasms, such as psammomatous melanotic schwannomas.

Resection of a dumbbell-shaped thoracic neurinoma by hemilaminectomy: a case report.

Neurinomas originating from intercostal nerve roots can grow both inside and outside of the spinal canal, forming dumbbell-shaped tumors. Such a neurinoma was discovered at the Th3 and Th4 levels in a 73-year-old woman during evaluation for breast cancer surgery. Magnetic resonance images (MRI) showed spinal cord compression by the tumor despite lack of neurologic symptoms. The tumor was resected successfully via hemilaminectomy with costotransversectomy. Postoperative course was uneventful, and no stabilization was needed after operation. Back pain was the only postoperative complication. Analgesics were administered for 1 month, and the pain resolved over 3 months. No recurrent neurinoma was found in follow-up images at 8 months. We consider hemilaminectomy safe and effective for complete resection of a dumbbell-shaped thoracic neurinoma.

Molecular markers of miscellaneous primary and metastatic tumors of the uterine cervix.

Miscellaneous primary tumors of the uterine cervix are rare. Markers which can be utilized to detect these tumors are very few and in most cases, have not been clinically validated. The information provided in this article will help in developing strategies to discover novel markers and initiate translational research in this ignored area. Based on the reported studies, cytokeratin markers are common in many tumors and few of these rare cancers demonstrate human papilloma-virus (HPV) and Epstein Bar virus (EBV) infection. Due to the very low prevalence of these tumors, epidemiological studies have not been conducted and the etiology of these tumors is largely unknown.

Spindle cell type malignant peripheral nerve sheath tumor arising in benign schwannoma with multiple intraosseous spinal metastasis: A case report.

BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) arising in benign schwannoma with multiple intraosseous spinal metastasis is extremely rare, having a highly aggressive progression and poor prognosis. In such cases, the malignant cells of MPNST usually have an epithelioid morphology. Here, the authors present a very rare case of spindle cell type MPNST arising in benign schwannoma. CASE: A 47-year-old woman had a history of wide marginal excision of right buttock spindle cell sarcoma previously. However, metastatic lesions to C7, L1 body, and the right lung were detected during follow-up. Total spondylectomy and stabilization of the C7 and L1 tumors were performed within an interval of 5 months. However, the patient expired 6 months after the last surgery. From analysis and study of three tumor specimens (right buttock, cervical and lumbar spine), the pathological diagnosis based on histomorphologic and immunohistochemical studies was spindle cell sarcoma, high grade, most consistent with MPNST arising in schwannoma. RESULTS: It is important that pathologists and surgeons recognize that spindle cell type MPNST may arise in benign schwannoma, as this recognition aids in assessment of patients with schwannoma and contributes to the pathologist making a more precise diagnosis, and the surgeon better determining the appropriate therapeutic options and surgical methods.

Low-grade Schwann cell neoplasms with leptomeningeal dissemination: clinicopathologic and autopsy findings.

Leptomeningeal dissemination of low-grade Schwann cell neoplasms is an exceptionally rare occurrence and has not been well documented in the literature. We encountered 2 cases of leptomeningeal dissemination of low-grade Schwann cell neoplasms. Patient 1 was a 63-year-old woman with neurofibromatosis type 1 and a progressive low-grade malignant peripheral nerve sheath tumor developing from a diffuse/plexiform orbital neurofibroma that arose in childhood. The neoplasm demonstrated local and leptomeningeal dissemination intracranially leading to the patient's death. There was partial loss of H3K27 tri-methylation, p16 and collagen IV. Patient 2 was a 60-year-old man without neurofibromatosis type 1 who presented with cranial nerve symptoms and a disseminated neoplasm with a Schwann cell phenotype. The neoplasm stabilized after irradiation and chemotherapy, but the patient died of medical complications. Autopsy findings documented disseminated leptomeningeal disease in the intracranial and spinal compartment. H3K27M tri-methylation was preserved. The clinicopathologic and autopsy findings are studied and presented, and the literature is reviewed.

Gamma Knife surgery for multiple brain metastases from a malignant schwannoma of the penis.

The occurrence of brain metastases from a malignant schwannoma of the penis is extremely rare. In patients with a single brain metastasis, microsurgical extirpation is the treatment of choice and verifies the diagnosis. In cases of multiple or recurrent metastases, radiosurgery is an effective and safe therapy option. Gamma Knife surgery was performed in a patient who had previously undergone tumor resection and who presented with recurrence of the lesion and three de novo brain metastases. This first report on brain metastasis from a malignant penile schwannoma illustrates the efficacy and safety of radiosurgical treatment for these tumors.

Giant malignant peripheral nerve sheath tumor of the scalp.

Herein, we describe a rare case of giant malignant peripheral nerve sheath tumor of the head in a 38-year-old Japanese man. The tumor measured 210 mm at its largest diameter and was ulcerated, hemorrhagic, multilocular and non-mobile. It should be noted that the patient stubbornly refused to see a doctor for a long time, resulting in the extreme growth of the tumor. We suspect a psychological basis for this behavior. Dermatohistopathological findings of the biopsy indicated ancient schwannoma and total excision was therefore performed. However, after 4 months, the patient developed multiple metastases and died. Post-mortem skin biopsy revealed features of malignant peripheral nerve sheath tumor. We performed immunohistochemical studies on the primary and recurrent lesions and concluded that there was a difference in the expression of Ki67 and p16. We propose that the expressions of Ki67 and p16 should be checked for all lesions of peripheral nerve sheath tumor for distinguishing benign from malignant forms.