Spinal extranodal Rosai-Dorfman disease: A case report and literature review.

INTRODUCTION: Spinal extranodal Rosai-Dorfman disease (RDD) is extremely rare. In this paper, we reported successful management of spinal extranodal RDD and reviewed medical literature. CASE PRESENTATION: A 19-year-old male presented with progressive bilateral leg weakness and back pain for two months before admission. He denied weight loss, fever, night sweats, and lymph node enlargement. On examination, his muscle strength of both legs was grade I with hyperreflexia. Magnetic resonance imaging of the spine (MRI) showed a thoracic extradural mass at a level of T6-T9, which was a heterogeneous hyperintense on T2W, STIR, and isointense on T1W and enhanced contrast vividly. We resected the tumor totally and decompressed the spinal cord. Pathology revealed a histiocytic tumor. Immunohistochemical staining was S100 (+), CD68 (+), CD45 (+), and CD1a (-). Postoperatively, his muscle strength improved gradually to grade IV after four months. Postoperative MRI of the spine showed no residual tumor. No further adjuvant therapy was indicated. CLINICAL DISCUSSION: Spinal extranodal RDD has no specific symptoms and pathognomonic imaging features. CT and MRI of the spine are still the essential tools for diagnosing RDD, but biopsy is often mandatory for definitive diagnosis. There have not been consensus guidelines for treating RDD of the spine because of its rarity. Surgical resection remained the mainstay of treatment (78.8%), with or without adjuvant therapies. CONCLUSION: Surgery is the treatment of choice for most cases, while steroid therapy, radiotherapy, and chemotherapy should be adjuvant treatment and tailored individually.

Arachnoid cyst with spontaneous intracystic hemorrhage and associated chronic subdural hematoma: A case report.

INTRODUCTION AND IMPORTANCE: Spontaneous hemorrhage of the arachnoid cyst was rare, especially associated with chronic subdural hematoma (CSDH). In this paper, we reported successful surgical management of arachnoid cyst with spontaneous hemorrhage and associated subdural hematoma. CASE PRESENTATION: A 33-year-old female with no medical history was presented with a headache for one month prior to admission. Head computed tomography and magnetic resonance imaging showed a left hypodense middle cranial fossa arachnoid cyst and ipsilateral CSDH. The multiple-slice computed tomography with contrast showed no vascular abnormality. The patient was indicated for surgical hematoma evacuation, membranectomy, and fenestration of the arachnoid cyst. At the one postoperative month, the computed tomography showed a middle fossa arachnoid cyst with no hemorrhage. Until a postoperative year, the patient had no headache and no neurological deficits. She returned to daily activities and her work. CLINICAL DISCUSSION: This event's pathogenesis was thought of as a result of tearing of the outer wall of an arachnoid cyst. The most common cause was mild head trauma; however, spontaneous rupture of the cyst wall also occurred. Surgery was the most common and effective treatment. Evacuation of CSDH was mandatory, but the strategies treatment for arachnoid cysts varied. Surgical options for arachnoid cyst included endoscopic/microsurgical fenestration, membranectomy, and even the cysto-peritoneal shunting. CONCLUSION: Arachnoid cyst with spontaneous intracystic hemorrhage accompanying CSDH was an uncommon condition. Surgery was the most common and effective treatment. Besides evacuation of CSDH, endoscopic/microsurgical fenestration or membranectomy was recommended to prevent the recurrence.

Late recurrence of primary cerebellar germinoma at unusual site after complete response to radiotherapy.

BACKGROUND: The primary cerebellar germinoma is exceptional and difficult to diagnose preoperatively. Its recurrence at the middle cranial fossa after complete response to radiotherapy is unique and associated with a poor prognosis. This article aims to report the successful management of the late recurrence of primary cerebellar germinoma at an unusual site after 4 years of complete response to radiotherapy. CASE DESCRIPTION: A 22-year-old male was admitted to our hospital with complaints of severe headache and loss of balance. Brain magnetic resonance imaging (MRI) showed a triventricular hydrocephalus due to a 45x50mm cerebellar mass. Our preliminary diagnosis was medulloblastoma. First, we placed a ventriculoperitoneal shunt with the medium-pressure valve, and then we used midline suboccipital craniotomy to remove the tumor completely. The histopathology was germinoma. The patient received 24 Gy craniospinal irradiation (CSI) with a 16 Gy boost to the primary site and had an MRI follow-up every six months. After a 4-year follow-up, he complained of recurrent severe headaches. The brain MRI illustrated a 62 × 61 mm temporal mass. We extirpated this tumor, and histopathology again revealed germinoma. After that, the patient received induction radiotherapy. The 1-year postoperative MRI showed no tumor remnant. At the time of writing, the patient had no headache and no neurological deficits. CONCLUSION: Regular follow-ups with routine neuroaxis MRI should be recommended to detect recurrence early for all patients with intracranial germinomas. Surgical resection, if possible, and subsequent CSI are the most effective salvage treatment for recurrent germinoma.

Malignant mucosal melanoma of paranasal sinuses: A case report.

INTRODUCTION: Mucosal melanoma of paranasal sinuses is a rare disease with a challenging treatment and a poor prognosis. In this paper, we reported the successful multimodality treatment of malignant mucosal melanoma of frontal sinus. CASE PRESENTATION: A 65-year-old female presented with a frequent nosebleed for one month before admission. Computed tomography and magnetic resonance imaging showed a mass in the right frontal and ethmoidal sinuses with adjacent bone erosion and right orbit invasion. Biopsy revealed malignant melanoma. No metastasis was found. The definitive diagnosis was malignant mucosal melanoma of paranasal sinuses AJCC stage IVb (T4bN0M0). We used right frontobasal craniotomy to resect tumor for local control of the disease. Immunohistochemical staining was Melan A(+), S100(+), and HMB45(+). A week postoperative, she received adjuvant radiotherapy and immunotherapy (pembrolizumab). For three months postoperative, the patient had no recurrence and metastasis, no headache and no new neurological deficits. She returned to her daily activities. CLINICAL DISCUSSION: Mucosal melanoma of paranasal sinuses is usually aggressive and diagnosed at an advanced stage. Management options are surgery, radiation therapy, chemotherapy, and immunotherapy. These options were performed on a case-by-case basis and depend on the extent and location of the tumor. Despite that, the prognosis remains very poor, with a high rate of local recurrences and distant metastases. Therefore, post-treatment lifetime and frequent follow-ups are highly recommended. CONCLUSION: The critical issues in management of mucosal melanoma are early diagnosis, multimodality treatment, and frequent follow-ups.

Acute spontaneous subdural hematoma secondary to ruptured arteriovenous malformation: A rare entity.

INTRODUCTION: Acute spontaneous subdural hematoma (ASSDH) due to ruptured arteriovenous malformation (AVM) is exceptional. There were only four reported cases. In this paper, we present a successful multimodality treatment of the ASSDH secondary to ruptured AVM. CASE PRESENTATION: A 21-year-old healthy man with no history of trauma presented to our hospital with complaints of severe headache for 12 hours before admission. On examination, he was alert and oriented. He had no intracranial hypertension, meningismus, and neurological deficits. Computed tomography illustrated a right acute subdural hematoma 8mm in thickness with a 5mm midline shift and a right frontal intraparenchymal hemorrhage 40 × 25mm in size. Digital subtraction angiography showed a 2 × 3 cm right frontal AVM, Spetzler-Martin grade I. The feeding arteries were cortical branches of the right anterior cerebral artery, and drain veins were cortical veins. He received emergency preoperative embolization followed by hematoma evacuation and total excision of the malformation. His headache was relieved and disappeared after a week. No postoperative neurological deficits were reported. CLINICAL DISCUSSION: Elective surgical resection of AVM after 4-6 weeks was preferred in patients with no risk factors of rebleeding. Emergent surgery was only indicated for significant mass effect or acute hydrocephalus. Preoperative embolization is helpful for the presence of intra-nidal or peri-nidal aneurysm, AVM with high grades, reducing intraoperative blood loss and occlusion of deep vessels. CONCLUSION: ASSDH due to ruptured AVM is rare and easy to omit in clinical settings. Preoperative embolization and surgical excision are effective treatments.

Surgical management of cerebral peduncle neurocysticercosis: A case report of decision making in a resource-restricted environment.

BACKGROUND: Diagnosis and treatment of neurocysticercosis have always been a challenge, especially in Vietnam, a resource-restricted environment. Here, we reported successful surgical management of cerebral peduncle neurocysticercosis. PRESENTATION OF CASE: A 65-year-old man with no medical history complained of headache and progressive right-sided hemiparesis for one month prior to admission. On examination, his muscle strength was 3/5 (Frankel grade) with positive Hoffmann and Babinski's signs on the right side. His brain's magnetic resonance imaging suggested a cystic brain tumor (29 × 18 mm) situated in the left cerebral peduncle and hypothalamus region. Enzyme-linked immunoassays (ELISA) of serum for Cysticercus cellulosae, Echinococcus, Toxocara, and Amebiasis were negative. The operation was performed with the modified frontotemporal-orbitozygomatic approach. We extirpated both the cysts. Histopathological examination revealed the larva of Taenia solium. Postoperatively, the hemiplegia was completely recovered, and no complication was reported DISCUSSION: The most recent revised diagnostic criteria of neurocysticercosis emphasised that neuroimaging studies play the most crucial role while clinical manifestations and exposure criteria were only indirect proofs of neurocysticercosis. Neuroimaging and clinical evidence in this patient, however, were more suggestive of cystic brain tumor, which was our most likely preoperative diagnosis. Consequently, total tumor resection required for both histopathological examination and alleviating his hemiplegia. CONCLUSIONS: Preoperative diagnosis of neurocysticercosis has always been a challenge. Total resection of the cerebral peduncle neurocysticercosis was adequate when the patient had localized neurological deficits.

Long-term outcomes of rotating gamma knife for vestibular schwannoma: A 4-year prospective longitudinal study of 89 consecutive patients in Vietnam.

BACKGROUND: Microsurgical total removal of vestibular schwannoma (VS) is the definitive treatment but has a high incidence of postoperative neurological deficits. Rotating Gamma Knife (RGK) is a preferred option for a small tumor. This study aims to evaluate long-term neurological outcomes of RGK for VS. METHODS: This prospective longitudinal study was conducted at the Nuclear Medicine and Oncology Center, Bach Mai Hospital, Hanoi, Vietnam. Eighty-nine consecutive patients were enrolled from October 2011 to October 2015 and followed up to June 2017. RGK was indicated for VS measuring <2.2 cm, while RGK for tumors measuring 2.2-3 cm was considered in patients with severe comorbidities, high-risk surgery, and who denied surgery. Concurrently, VS consisted of newly diagnosed, postoperative residual, and recurrent tumors. Patients with neurofibromatosis type 2 were excluded from the study. Primary outcomes were radiological tumor control rate, vestibulocochlear functions, facial and trigeminal nerve preservation. Stereotactic radiosurgery was performed by the Rotating Gamma System Gamma ART 6000. RESULTS: The tumors were measured 20.7 ± 5.6 mm at pre treatment and 17.6 ± 4.1 mm at 3-year post treatment. The mean radiation dose was 13.5 ± 0.9 Gy. Mean follow-up was 40.6 ± 13.3 months. The radiological tumor control rate was achieved 95.5% at 5-year post treatment. The hearing and vestibular functions were preserved in 70.3% and 68.9%, respectively. The facial and trigeminal nerve preservation rates were 94.4% and 73.3%, respectively. CONCLUSION: RGK is an effective and safe treatment for VS measuring ≤3 cm with no significant complications during long-term follow-up.

Microsurgery for intradural epidermoid cyst at cauda equina level in a 9-year-old child: A case report.

INTRODUCTION AND IMPORTANCE: Epidermoid cysts are rare benign tumors. Here, we present a case of spontaneous intradural epidermoid cyst at cauda equina level in a 9-year-old patient, which we believed the first case to be reported in Vietnam. CASE PRESENTATION: A 9-year-old boy presented with 4 months of spontaneous left lower extremity muscle weakness and paresthesia. The MRI images suggested the diagnosis of intradural epidermoid cyst at cauda equina level. The patient underwent L5-S1 laminectomy and durotomy for tumor resection. The histology confirmed the diagnosis of epidermoid cyst. Post-operative images demonstrated total cyst removal. CLINICAL DISCUSSION: The epidermiology, presentation and diagnosis and strategy of treatments as well as their outcomes were discussed. CONCLUSION: Diagnosis of spinal epidermoid cyst is often delayed for its obscure presentation. Microsurgical dissection along with intra-operative mobile C-Arms enable total tumor resection while preserving spinal stability and neurological function. Follow-up with post-operative magnetic resonance imaging and tumor marker are helpful.

Spontaneous cranial bone regeneration following craniectomy for traumatic brain injury in a pregnant woman: A case report.

INTRODUCTION AND IMPORTANCE: Spontaneous bone formation following craniectomy is an extremely rare in adult. As in the medical literature, this is the first case report on total spontaneous ossification following craniectomy in a pregnant woman. CASE PRESENTATION: In this paper, we reported a 20-year-old female currently in the 30th week of her pregnancy suffered from head trauma following motorcycle accident. On admission to our hospital, her GCS score was 3 points. She was treated with emergency extradural hematoma evacuation with craniectomy and Caesarean section with uterine artery ligation. 3 weeks post-operation, the patient and her daughter were discharged from the hospital. At follow-up, spontaneous cranial bone generation was observed. CLINICAL DISCUSSION: The presentation, diagnosis and strategy of treatments were discussed. CONCLUSION: Diagnostic imaging in traumatic pregnant patient is often postponed for the concern of fetus exposure to radiation. Traumatic pregnant patient with possible head trauma should be transferred to a center with expertise in neurotrauma and obstetrical care. Spontaneous cranial bone regeneration following craniectomy in adult is rare. Surgery techniques and hormones in pregnancy contribute to bone formation.

Endoscopic-assisted microvascular decompression for trigeminal neuralgia secondary to vertebrobasilar dolichoectasia: A case report.

INTRODUCTION AND IMPORTANCE: Trigeminal neuralgia (TN) secondary to vertebrobasilar dolichoectasia (VBD) was a rare condition. This paper reported a successful endoscopic-assisted microvascular decompression (MVD) for TN secondary to VBD. CASE PRESENTATION: A 53-year-old male with a history of myocardial infarction and heart failure complained of left refractory facial pain three years prior to admission. His pain was intermittent, electrical-like, severe, sharp, and radiated along the maxillary and mandibular branches. He used carbamazepine 600 mg daily. Brain magnetic resonance imaging revealed a neurovascular conflict between VBD and the left trigeminal nerve. The endoscopic-assisted MVD was indicated. The shredded neurosurgical sponges were interposed between VBD and trigeminal nerve. The 0° and 30° rigid rod-lens endoscope was used to explore and confirm the complete decompression. Postoperatively, TN was disappeared entirely. At a three-month postoperative, no facial pain and paraesthesia were found. The patient discontinued carbamazepine permanently. CLINICAL DISCUSSION: MVD was still the most effective treatment. An inspection of root entry zone (REZ) and complete MVD with a solely operating microscope were challenging due to the massive diameter of VBD and multiple offending arteries behind the VBD. However, the wide viewing field and high-quality resolution of endoscopes allowed better visualization of REZ and neurovascular conflicts behind neural structures and least cerebellar retraction. This is essential in case of less potential space created by VBD. CONCLUSION: Endoscope-assisted MVD allowed better visualization of REZ and neurovascular conflicts behind neural structures and least cerebellar retraction in management of trigeminal neuralgia secondary to VBD.

Infected intradural dermoid cyst without dermal sinus tract mimicking brain abscess: A case report.

INTRODUCTION: Infection is a rare complication of intradural dermoid cyst. We reported an infected intradural dermoid cyst without dermal sinus tract mimicking brain abscess. PRESENTATION OF CASE: A 4-year-old boy with no medical history complained of a palpable mass on his head. On examination, the occipital palpable mass was firm and immobile, had no redness, swelling, and pain. The preoperative MRI showed a well-defined, ring-enhancing lesion. White blood count was 12.8 × 109  cells/liter. We have sought no other infection sites. We encountered intraoperatively the pus from the infected mass invading subcutaneous layer and skull bone. We excised completely the tumor and carefully coagulated the residual capsule invading superior sagittal sinus. Histopathological examination was infected dermoid cyst. The infection agent was Staphylococcus aureus. The patient was received systemic antibiotic therapy for 21 days following oral antibiotics for 1 month. He was discharged with no complications. DISCUSSION: The diagnosis of infected dermoid cyst was often based on MRI images and especially dermal sinus tract. However, in the absence of a dermal sinus tract, preoperative diagnosing an infected dermoid cyst might be very challenging. The ideal treatment of the dermoid cyst was total resection of the cyst with the epithelial lining. Nevertheless, due to the benign nature of dermoid cyst, adequate gross total resection with meticulous hemostasis the residual capsule firmly adhering eloquent areas and major vessels was more appropriate. CONCLUSION: Diagnosing infected dermoid cyst with no dermal sinus might be challenging. Systemic antibiotic therapy after gross total resection was an effective treatment.

STA-MCA bypass following sphenoid wing meningioma resection: A case report.

INTRODUCTION: Sphenoid meningioma engulfed cerebral arteries has always been a challenge. To achieve a gross total resection, vessel sacrifice may be unavoidable. PRESENTATION OF CASE: A 22-year-old man with a history of head trauma a week ago complained of a headache for one week. On examination, he was alert, denied paralysis and cranial nerves palsies. Preoperative MRI showed a hypervascular left sphenoid wing meningioma embedding left internal carotid artery and proximal segment of the middle cerebral artery. In operation, a branch of the MCA was divided when dissecting the tumor. The MCA was clipped but was still difficult to dissect vessel ends in the Sylvian fissure. We decided to extend craniotomy and did superficial temporal artery to M4 segment of MCA bypass. Then, the patient was resuscitated in surgical high dependency unit for 3 days. Surgical outcome in one year postoperative was good with KPS 90 out of 100 points and no neurological deficits. On postoperative MRA, STA-MCA bypass shown acceptable flow. DISCUSSION: There were a few cases of skull base tumors requiring vessel revascularization. Most of the revascularization cases were meningiomas. Saphenous vein graft (SVGs) was the most commonly reported graft, followed by radial artery graft (RAGs). In case of difficulty in dissecting the vessel ends due to the tumor infiltration, STA-MCA bypass was a safe and helpful choice, especially the collateral vessels were present and the need for blood flow augmentation was minimal. CONCLUSION: STA-MCA bypass was effective surgical management for MCA injury in sphenoid wing meningioma resection.