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Plexiform schwannoma is a rare subtype of schwannoma. In this report, we present a case of plexiform schwannoma arising from the sciatic, tibial, and peroneal nerves. A 54-year-old man presented with a painful palpable mass extending from the left posterior thigh to the calf. Magnetic resonance imaging showed multiple bead-like nodular structures along the sciatic, tibial, and peroneal nerve pathway. The nodular lesions showed uniform signal intensity on T1-weighted imaging. On T2-weighted imaging, each nodule showed an eccentric area of relatively low signal intensity surrounded by an area of higher signal intensity and a low-intensity rim. Plexiform schwannoma or neurofibroma was considered as the preoperative diagnosis. Because of the patient's severe symptoms and strong desire for relief, tumor enucleation of the largest painful nodule was performed, and plexiform schwannoma was confirmed pathologically.

A plexiform schwannoma is a subtype of schwannoma characterized by a multinodular and plexiform pattern with a superficial occurrence, mainly under the skin or subcutaneous tissue. It is rarely located deep or occurs in the major peripheral nerves. In this case, it is difficult to distinguish a plexiform schwannoma from a malignant peripheral schwannoma, so an excisional biopsy is required. On the other hand, there are no reports on the long-term follow-up of surgical treatment performed on major peripheral nerves. The authors report the confirmation of a plexiform schwannoma on the right ulnar nerve in a 64-year-old male patient by excisional biopsy and long-term observations for seven years. [ABSTRACT FROM AUTHOR]

Plexiform schwannoma is an uncommon benign tumour that grows in a plexiform pattern. We report a 47-year-old man with a mass on the palmar aspect of the metacarpophalangeal joint of the right index finger that had been growing gradually for more than 10 years. The mass was palpated from the distal carpal tunnel to the ulnar aspect of the proximal interphalangeal joint of the index finger, with tingling and numbness sensation. The tumour was a multinodular tumour involving the first common palmar digital nerve to the ulnar proper palmar digital nerve. It was resected and reconstructed with a sural nerve graft. Plexiform schwannoma is rare in the digital nerve, with only six cases reported. Generally, classic schwannomas can be enucleated without causing neurologic deficits; however, plexiform schwannoma may require nerve resection. There have been reports of recurrence of plexiform schwannoma; definitive resection and long-term follow-up are necessary. Level of Evidence: Level V (Therapeutic) [ABSTRACT FROM AUTHOR]

Plexiform schwannoma (PS) is a rare tumor that accounts for only 5 % of all schwannomas. Most cases are small, measuring less than 3 cm in diameter, and involve the skin and superficial tissue of the head, neck, or trunk. The location of the pelvis is extremely rare for PS and even so for ordinary schwannomas. We report on our case of a four-year-old Arabic boy who presented with a palpable mass in the perineum. This was found to represent an extrapelvic extension of a large intrapelvic plexiform schwannoma measuring 8 cm in maximum dimension on magnetic resonance imaging (MRI). A diagnosis of plexiform neurofibroma was suspected. However, the mass was surgically resected and histologically confirmed to be benign PS. The location of the pelvis is extremely rare for PS and this clinical presentation with a perineal mass is unique to our case. Distinguishing PS from plexiform neurofibroma or malignant peripheral nerve sheath tumor is of utmost clinical importance. However, the preoperative diagnosis is often challenging due to nonspecific imaging features. The MRI findings highlighted here may help in the preoperative diagnosis of this rare pelvic tumor.

Abstract Background Plexiform schwannoma is one of the least common variants of schwannoma, accounting for only 5% of all schwannoma cases. It generally occurs in the skin and subcutaneous tissues and is uncommon in deep soft tissue or viscera. We present an extremely rare case of plexiform schwannoma arising from the peripancreatic plexus. Case presentation A 29-year-old man presented with hyperglycemia detected during a medical checkup. He was diagnosed with type 1 diabetes based on the clinical findings and laboratory tests. During the diagnostic process for diabetes, a 2.5 cm mass was incidentally detected in the pancreas by abdominal ultrasound. Contrast-enhanced computed tomography revealed a mass that was gradually enhanced at the body and tail of the pancreas. Magnetic resonance imaging revealed low signal intensity of the mass on T1-weighted images and high signal intensity on T2-weighted and diffusion-weighted images. Magnetic resonance cholangiopancreatography showed no abnormal findings in the main pancreatic duct. Endoscopic ultrasonography (EUS) showed a lobulated, low-echoic mass with a clear boundary. EUS-guided fine needle biopsy was performed, and spindle-shaped cells that were diffusely immunopositive for S-100 and negative for c-kit and desmin were detected, resulting in a diagnosis of a neurogenic tumor arising from the pancreas or the peripancreatic nerve plexus. The patient underwent laparoscopic spleen-preserving distal pancreatectomy. Although the tumor was connected to the splenic plexus, the splenic artery could be divided along its adventitial plane. Macroscopic findings of the excised tumor consisted of multiple yellowish-white nodules, and its histopathological features were consistent with plexiform schwannoma. There was no pancreatic tissue on the dorsal surface of the tumor, which suggested that the tumor arose from the peripancreatic nerve plexus. Conclusions The findings documented herein can aid in the differential diagnosis of peripancreatic schwannoma and in planning appropriate treatment.

Abstract Background Plexiform schwannoma (PS), variant of schwannoma, often involves multiple fascicles as plexiform neurofibroma, and is usually located superficially on the dermis and subcutaneous layers. Spinal PS is extremely rare, and there is insufficient information on its natural course and treatment strategy. We describe the clinical features and treatment of giant intradural PS at the lumbosacral spine. Case presentation A 66-year-old man presented with leg pain, paresthesia, and weakness for 2 years. Magnetic resonance imaging demonstrated a large mass lesion involving a continuous multi-lobulated bead-like mass and a cystic portion from L1 to S3. The lesion was iso-intense on T2-weighted images (WI), iso- to slightly low-intense on T1-WI, and heterogeneous enhancement on contrast-enhanced T1-WI. The large mass lesion had three portions, including a cystic mass at L1, continuous multi-lobulated bead-like mass with a cystic portion from L2 to S1, and multi-lobulated mass from S2 to S3, which were identified with severe adhesions with cauda equina on operative assessment. Grossly total extirpation was achieved at the lumbar spine, and remained three round shaped small masses at the lumbar area and a multi-lobulated round masses from S2 to S3 involving nerves related with motor function of the lower extremities and anal sphincter, respectively. Histological examination revealed multinodular or plexiform growth pattern composed of spindle-shaped tumor cells, which were diffusely and strongly positive for S100 protein with KI67

Background: Plexiform schwannomas (PSs) are rare. Here, we describe the clinical features, diagnosis, treatment, and outcome of a 17-year-old male presenting with a T1-T4 intradural extramedullary (IDEM)., Case Description: A 17-year-old male presented with back pain and pain radiating down both legs. The thoracic magnetic resonance revealed a left-sided T1-T4 IDEM mass. The patient underwent a T2-T4 laminectomy for gross total tumor excision, followed by posterior fusion. Immunohistochemical examination revealed S100 positivity, supporting the diagnosis of PS., Conclusion: IDEM PSs are rare, may be readily diagnosed with MR, and can be successfully resected., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Surgical Neurology International.)

Background: Plexiform schwannoma is one of the least common variants of schwannoma, accounting for only 5% of all schwannoma cases. It generally occurs in the skin and subcutaneous tissues and is uncommon in deep soft tissue or viscera. We present an extremely rare case of plexiform schwannoma arising from the peripancreatic plexus. Case presentation: A 29-year-old man presented with hyperglycemia detected during a medical checkup. He was diagnosed with type 1 diabetes based on the clinical findings and laboratory tests. During the diagnostic process for diabetes, a 2.5 cm mass was incidentally detected in the pancreas by abdominal ultrasound. Contrast-enhanced computed tomography revealed a mass that was gradually enhanced at the body and tail of the pancreas. Magnetic resonance imaging revealed low signal intensity of the mass on T1-weighted images and high signal intensity on T2-weighted and diffusion-weighted images. Magnetic resonance cholangiopancreatography showed no abnormal findings in the main pancreatic duct. Endoscopic ultrasonography (EUS) showed a lobulated, low-echoic mass with a clear boundary. EUS-guided fine needle biopsy was performed, and spindle-shaped cells that were diffusely immunopositive for S-100 and negative for c-kit and desmin were detected, resulting in a diagnosis of a neurogenic tumor arising from the pancreas or the peripancreatic nerve plexus. The patient underwent laparoscopic spleen-preserving distal pancreatectomy. Although the tumor was connected to the splenic plexus, the splenic artery could be divided along its adventitial plane. Macroscopic findings of the excised tumor consisted of multiple yellowish-white nodules, and its histopathological features were consistent with plexiform schwannoma. There was no pancreatic tissue on the dorsal surface of the tumor, which suggested that the tumor arose from the peripancreatic nerve plexus. Conclusions: The findings documented herein can aid in the differential diagnosis of peripancreatic schwannoma and in planning appropriate treatment. [ABSTRACT FROM AUTHOR]

Background: Plexiform schwannoma (PS), variant of schwannoma, often involves multiple fascicles as plexiform neurofibroma, and is usually located superficially on the dermis and subcutaneous layers. Spinal PS is extremely rare, and there is insufficient information on its natural course and treatment strategy. We describe the clinical features and treatment of giant intradural PS at the lumbosacral spine.Case Presentation: A 66-year-old man presented with leg pain, paresthesia, and weakness for 2 years. Magnetic resonance imaging demonstrated a large mass lesion involving a continuous multi-lobulated bead-like mass and a cystic portion from L1 to S3. The lesion was iso-intense on T2-weighted images (WI), iso- to slightly low-intense on T1-WI, and heterogeneous enhancement on contrast-enhanced T1-WI. The large mass lesion had three portions, including a cystic mass at L1, continuous multi-lobulated bead-like mass with a cystic portion from L2 to S1, and multi-lobulated mass from S2 to S3, which were identified with severe adhesions with cauda equina on operative assessment. Grossly total extirpation was achieved at the lumbar spine, and remained three round shaped small masses at the lumbar area and a multi-lobulated round masses from S2 to S3 involving nerves related with motor function of the lower extremities and anal sphincter, respectively. Histological examination revealed multinodular or plexiform growth pattern composed of spindle-shaped tumor cells, which were diffusely and strongly positive for S100 protein with KI67 < 1%. There were no recurrence of preoperative symptoms and changes of the remained masses over a 2-year follow-up period.Conclusion: Subtotal extirpation to minimize neural deficits and close observation can be considered an appropriate treatment strategy for a giant spinal PS considering its benign prognosis and histological features, with a high risk of neurological damage during surgery. [ABSTRACT FROM AUTHOR]

Neurofibromatosis type II (NF2) is a tumor predisposition syndrome characterized by the development of distinctive nervous system lesions. NF2 results from loss-of-function alterations in the NF2 gene on chromosome 22, with resultant dysfunction of its protein product merlin. NF2 is most commonly associated with the development of bilateral vestibular schwannomas; however, patients also have a predisposition to development of other tumors including meningiomas, ependymomas, and peripheral, spinal, and cranial nerve schwannomas. Patients may also develop other characteristic manifestations such as ocular lesions, neuropathies, meningioangiomatosis, and glial hamartia. NF2 has a highly variable clinical course, with some patients exhibiting a severe phenotype and development of multiple tumors at an early age, while others may be nearly asymptomatic throughout their lifetime. Despite the high morbidity associated with NF2 in severe cases, management of NF2-associated lesions primarily consists of surgical resection and treatment of symptoms, and there are currently no FDA-approved systemic therapies that address the underlying biology of the syndrome. Refinements to the diagnostic criteria of NF2 have been proposed over time due to increasing understanding of clinical and molecular data. Large-population studies have demonstrated that some features such as the development of gliomas and neurofibromas, currently included as diagnostic criteria, may require further clarification and modification. Meanwhile, burgeoning insights into the molecular biology of NF2 have shed light on the etiology and highly variable severity of the disease and suggested numerous putative molecular targets for therapeutic intervention. Here, we review the clinicopathologic features of NF2, current understanding of the molecular biology of NF2, particularly with regard to central nervous system lesions, ongoing therapeutic studies, and avenues for further research. [ABSTRACT FROM AUTHOR]

Schwannoma, a benign nerve sheath tumor, is quite rare and more so in jawbones. We report a rare case of a plexiform variant of mandibular schwannoma in a 12-year-old female with a swelling in the left mandible. Clinical features were suggestive of dentigerous cyst as a result of missing premolars and canine. Occlusal and panoramic radiography revealed an osteolytic lesion with scalloping margins, bicortical plate expansion, and agenesis of several teeth. Odontogenic keratocyst, central giant cell granuloma, odontogenic myxoma, and ameloblastic fibroma were given as radiological differential diagnoses. Histopathological examination revealed features of plexiform schwannoma which was given as the final diagnosis. The lesion was treated with surgical excision. Although odontogenic cysts/tumors are often thought of in differential diagnosis whenever well-defined radiolucencies in the jaw are encountered, it is prudent to include schwannoma. This exceptional case adds light to the fact that schwannoma should not be overlooked though it is a rare possibility and must be included in differential diagnosis of odontogenic cysts/tumors. [ABSTRACT FROM AUTHOR]

Multiple schwannomatosis, is defined as two or more, pathologically proven schwannomas, without radiographic evidence of vestibular nerve tumor, below 18 years, or absence of VIII nerve dysfunction beyond 30 years with absence of stigmata of neurofibromatosis 2 (NF2). The plexiform schwannoma (PS) is a rare tumor. The gross appearance is an important diagnostic criterion for diagnosis of PSs. It is associated with NF2, which was ruled out in this case. Here we present a 50 year female patient with two nonintradermal schwannomas, in the supraclavicular and infraauricular deep soft tissues. One was plexiform, diagnosed on cytology and confirmed by histopathology. She also had multiple pulmonary and pleural lesions. Fine needle aspiration cytology smears from both supraclavicular and infraauricular lesions as well as the lung lesion sampled, showed spindle cells in a myxoid background, having wavy nuclei with pointed ends. Verocay body - like palisading of nuclei was noted.

Plexiform schwannoma (PS) is an uncommon variant of schwannoma characterized by a multinodular (plexiform) growth pattern. It comprises up to 5 % of all schwannomas. The association between PS and neurofibromatosis type 1 or type 2 (NF1/NF2) is only rarely reported. Most cases of PS occur in the skin and subcutaneous soft tissue, with only a few reports of digestive tract involvement. We describe an 18-year-old male with NF2 who had bilateral vestibular schwannomas and multiple cutaneous PSs, and a 3-year history of abdominal pain. The patient ultimately underwent a distal gastrectomy for a partially obstructing submucosal antral mass, associated with an overlying ulcer. Histopathologic examination showed the mass to be a PS. The patient is alive and well, without symptoms, 12 months postoperatively. A review of the English language medical literature yielded only ten examples of PS arising in the digestive tract. Our patient is the first to be reported to have a gastric PS, and only the second patient to be reported with a digestive tract PS to have NF2, and the only patient reported to have both digestive tract and cutaneous PSs. Despite its rare occurrence with NF2, the finding of PS at any site should stimulate an examination for other manifestations of this disorder. Clinical Trial Registration: None. [ABSTRACT FROM AUTHOR]

Rare epithelial structures in benign nerve sheath tumors are almost always glandular in appearance. We describe a case of intraoral plexiform schwannoma with concurrent squamous epithelial hyperplasia. The lesion occurred as a pigmented nodule on the gingiva of a 35-year-old woman with no systemic involvement. Histologically, unencapsulated, plexiform fascicular proliferations of schwann cells could be traced from the submucosa to the lamina propria, finally making direct contact with heavily pigmented, elongated rete ridges of the overlying epithelium. Also noted was a schwannian network centered on clustered odontogenic epithelial rests of mature squamous-type, the number and size of which had markedly increased. Impressive immunoprofiles of periepithelial neural microfascicles included the complete absence of axon and perineurium and the unexpected presence of endoneurial fibroblasts. The repertoire of epithelial changes was in a confined area with no extension beyond, supporting hyperplasia induction by an underlying/surrounding schwannoma. [ABSTRACT FROM AUTHOR]

Background: Tumors composed of cells differentiating as both fibroblasts and histiocytes have been designated fibrous histiocytomas. Only a small percentage of these lesions behave in a malignant fashion, they are called malignant fibrous histiocytoma (MFH).The occurrence of MFH in membranous bones including the mandible is quite unusual. Involvement of the mandible accounts for only 3 % of all MFH bone lesions. Recent literature revealed only a few (30) cases of MFH involving the mandible but not a single case of MFH associated with pathological fracture of the mandible, probably the first such kind of case to support antecedent trauma as an initial proliferative response for its occurence. Case report: A rare case of MFH involving the mandible and submandibular glands with pathological fracture in a 14-year-old boy is presented with special emphasis on the poor prognosis even after prompt therapy, its controversial histogenesis, high malignant potential, high recurrence rate and tendency to metastasise. The patient was regularly followed up for 11 months post-operatively, during which patient complained of pain in the operated region after 8 to 9 months. Though there was no clinically obvious abnormality seen, the young boy died after 11 months. The clinical, surgical, radiographic and pathological features of this lesion are discussed. Discussion: Malignant fibrous histiocytoma, the most frequent soft tissue sarcoma of adulthood, was first described as a new malignant tumour by O'Brian and Stout in the 1960s and the details of the histopathological features of MFH were first described by Kempson and Kyriakos. Despite the frequency of diagnosis, MFH has remained an enigma as no true cell of origin has ever been identified. Treatment consists of surgical excision and in some cases chemotherapy and radiation. Early and complete surgical removal using wide or radical resection is indicated because of the aggressive nature of the tumor. The combination of infrequent occurrence, varied pathologic features, uncertain histogenesis, numerous subtypes and the many potential sites of presentation makes these tumors a challenge for the diagnostician, surgeon and oncologist. Close follow-up after treatment is important, as local recurrence is common and early metastasis to the lungs is also frequent, which are the reasons for high mortality rate in MFH. [ABSTRACT FROM AUTHOR]

Multiple schwannomatosis, is defined as two or more, pathologically proven schwannomas, without radiographic evidence of vestibular nerve tumor, below 18 years, or absence of VIII nerve dysfunction beyond 30 years with absence of stigmata of neurofibromatosis 2 (NF2). The plexiform schwannoma (PS) is a rare tumor. The gross appearance is an important diagnostic criterion for diagnosis of PSs. It is associated with NF2, which was ruled out in this case. Here we present a 50 year female patient with two nonintradermal schwannomas, in the supraclavicular and infraauricular deep soft tissues. One was plexiform, diagnosed on cytology and confirmed by histopathology. She also had multiple pulmonary and pleural lesions. Fine needle aspiration cytology smears from both supraclavicular and infraauricular lesions as well as the lung lesion sampled, showed spindle cells in a myxoid background, having wavy nuclei with pointed ends. Verocay body -- like palisading of nuclei was noted. [ABSTRACT FROM AUTHOR]

Schwannoma is rare peripheral nerve sheath tumor arising from Schwann cells. It is generally benign but may show malignant transformation. Perineal location of these tumors is very rare. We operated a 6-year-old boy with perineal mass, which turned out to be a schwannoma. We are presenting the description of the case and review of the relevant literature. [ABSTRACT FROM AUTHOR]

Background: Plexiform schwannoma (PS) is a rare, peripheral nerve sheath tumor arranged in a plexiform pattern. Case presentation: We report an unusual case of a 19-year-old woman, who complained of pain in the plantar aspect of the left foot. Magnetic resonance image (MRI) demonstrates three solitary nodules of varying sizes in the deep soft tissue of the plantar aspect of the foot that are homogeneously isointense to skeletal muscle on T1-weighted images and hyperintense on T2-weighted fat-suppressed images, especially the rim of the lesion. Subsequent pathological examination confirmed the diagnosis of PS. Conclusion: MRI characteristic plays an important role in detecting this rare lesion. A review of the literature on PS is also presented. [ABSTRACT FROM AUTHOR]

Plexiform schwannoma is a benign peripheral nerve sheath tumor composed exclusively of Schwann cells arranged in a plexiform pattern. Most plexiform schwannomas are skin tumors and visceral localization is very rare. To our knowledge, there are six cases localized in visceral organs. We describe herein the first known case of plexiform schwannoma of the rectum resected by endoscopic submucosal dissection ( ESD). A 77-year-old woman presented with a short history of anal pain. Sigmoidoscopy demonstrated a submucosal tumor, 20 mm in diameter, of the rectum below the peritoneal reflection ( Rb). We resected the tumorby ESD. The tumor consisted of multiple white nodules in the submucosal layer. Microscopic examination revealed that the tumor was composed mainly of Antoni A tissue, compatible with conventional schwannoma. Immunohistochemically, the tumor was positive for S-100, and negative for α-smooth muscle actin, c-kit and CD-34. No evidence of recurrence has been found in 2 years of follow up. [ABSTRACT FROM AUTHOR]

Background: Neoplasms of peripheral nerve in the head and neck region are of common occurrence, but origin in the oral and para-oral tissues is uncommon and they rarely occur centrally within the jaws. Schwannoma is a benign neoplasm originating from the neural sheath of peripheral soft tissues, but its occurrence within the jaw bones is most unusual. Plexiform schwannoma is a unique variant of Schwann cell tumours having plexiform pattern. Literature revealed only one case of plexiform schwannoma of the jaw bones, i.e. involving the mandible. Case report: In this report, we present the first documented case of intraosseous plexiform schwannoma of the maxilla, an extremely rare benign neurogenic tumour treated surgically. Discussion: Schwannoma is a benign neoplasm originating from the neural sheath of peripheral soft tissues, but to occur within the jaw bones is exceptional. Plexiform schwannoma is a rare variant of Schwann cell tumour having plexiform pattern of intraneural growth with multinodularity. Plexiform schwannoma is a benign neoplasm with no malignant potential, but recurrences are evident if excised incompletely. Plexiform schwannoma has similar clinical and histopathological features as that of plexiform neurofibroma which has high malignant potential; hence, it is imperative to correctly diagnose and differentiate this lesion as treatment modality of these two lesions differs. [ABSTRACT FROM AUTHOR]

Macrodactyly is a rare anomaly. Little is understood about the etiology and underlying mechanisms. To our knowledge, macrodactyly has not previously been associated with neurofibromatosis type 2. We present a case of macrodactyly of the small finger associated with a digital nerve plexiform schwannoma in a patient with neurofibromatosis type 2. [Copyright &y& Elsevier]

Abstract: Plexiform schwannomas are extremely rare in the oral cavity. We report here the case of a 3-year-old child with plexiform schwannoma located in the hard palate. The tumor presented as a slowly growing nodule of about 6 months duration, mimicking clinically a salivary gland tumor. No signs of recurrence were observed after one year of follow-up. Benign peripheral nerve and salivary gland tumors should be included in the differential diagnosis of nodular lesions occurring in children. [Copyright &y& Elsevier]

Abstract: Neurofibromatosis type 2 (NF2) is an autosomal dominant disorder characterized by bilateral vestibular schwannomas. The initial symptoms of NF2 are usually hearing loss and tinnitus caused by vestibular schwannomas. Although other intracranial, spinal, or skin tumors also occur in NF2, laryngeal lesions are very rare. We report a rare case of NF2 with laryngeal plexiform schwannoma as first symptom. A 38-year-old man presented with a smooth-surfaced submucosal supraglottic mass. Two round masses in the left chest wall and left supraclavicular fossa were noted incidentally during investigation of the laryngeal mass. Magnetic resonance imaging (MRI) findings for these masses were identical to those of the laryngeal mass. No typical symptoms related to NF2 were identified. Histologically, the laryngeal tumor represented plexiform schwannoma. We thus considered that the two round masses in the left chest wall and left supraclavicular fossa might also represent plexiform schwannomas. NF2 was suspected, as a high incidence of multiple plexiform schwannomas has been suggested for patients with NF2. MRI of brain lesions demonstrated bilateral vestibular schwannomas and multiple meningiomas. Finally, NF2 with laryngeal plexiform schwannoma was diagnosed. Recognizing that multiple plexiform schwannomas could be associated with NF2 is important. [Copyright &y& Elsevier]

Abstract: Schwannoma is a benign neoplasia of the peripheral nerve sheath. Its localization in the gastrointestinal tract, and in particular in the esophagus, is very rare. According to the existing literature esophageal schwannoma has been reported so far only in adult patients. We report the case of an 11 year old patient with neurofibromatosis, type 2, who underwent surgical excision of a plexiform schwannoma of the esophagus. [Copyright &y& Elsevier]

Summary: Angiosarcoma developing within a schwannoma is rare, and only 8 cases have been reported in the literature. Its association with a plexiform schwannoma has never been reported. We describe an epithelioid angiosarcoma arising in a plexiform schwannoma of the sciatic nerve in a 73-year-old man not known to have von Recklinghausen disease. Grossly, the tumor forms a long tubular mass with a multinodular growth pattern on the cut surface. Microscopically, these nodules display characteristics of a schwannoma, including Antoni A, Antoni B, and Verocay bodies. Adjacent to these nodules are high-grade anaplastic epithelioid cells growing in solid sheets, cords, and nests with focal vasoformative channels. The anaplastic cells are immunoreactive to CD31, CD34, and factor VIII related antigen, supporting their endothelial differentiation. Local recurrence and multiple pulmonary metastasis occurred 3 months after complete excision of the tumor. This case demonstrates the first occurrence of an epithelioid angiosarcoma in a deep-seated plexiform schwannoma with an aggressive clinical behavior. [Copyright &y& Elsevier]

Summary: We report a case of a 12-year-old boy who presented with a 1-year history of left facial swelling. During surgical exploration of the parotid gland, a plexiform schwannoma of the facial nerve was discovered. Subsequently other features of neurofibromatosis type 2 (NF2) were unravelled—bilateral intracanalicular facial schwannomas, and ependymoma of the spine. We detail the course that led to achieving this extremely rare association. This is the eighth case of plexiform schwannoma associated with neurofibromatosis type 2 reported to date, the first ever to involve a cranial nerve, and the third to present in the pediatric population. [Copyright &y& Elsevier]

Summary: Schwannoma is a slow-growing tumor that frequently occurs in the extremities, the trunk and the head region. Its occurrence in the breast is rare with only a few cases being reported. We present here the case of breast schwannoma in a 41-year-old woman who presented with a palpable mass in her right breast. This is the first report of breast schwannoma that showed massive exophytic growth with invasion of the skin, and it was initially presumed to be a breast cancer on preoperative mammography, ultrasonography and breast MRI examinations. Complete excision of the mass was done and pathology revealed a plexiform schwannoma. [Copyright &y& Elsevier]