Chiazo Amene

Hemorrhagic intramedullary solitary fibrous tumor of the conus medullaris: case report

The differential diagnosis of spinal tumors is guided by anatomical location and imaging characteristics. Diagnosis of rare tumors is made challenging by abnormal features. The authors present the case of a 47-year-old woman who presented with progressive subacute right lower-extremity weakness and numbness of the right thigh. Physical examination further revealed an extensor response to plantar reflex on the right and hyporeflexia of the right Achilles and patellar reflexes. Magnetic resonance imaging of the lumbar spine demonstrated an 8-mm intramedullary exophytic nodule protruding into a hematoma within the conus medullaris. Spinal angiography was performed to rule out an arteriovenous malformation, and resection with hematoma evacuation was completed. Pathological examination of the resected mass demonstrated a spindle cell neoplasm with dense bundles of collagen. Special immunostaining was performed and a diagnosis of solitary fibrous tumor (SFT) was made. SFTs are mesenchymally derived pleural neoplasms, which rarely present at other locations of the body, but have been increasingly described to occur as primary neoplasms of the spine and CNS. The authors believe that this case is unique in its rare location at the level of the conus, and also that this is the first report of a hemorrhagic SFT in the spine. Therefore, with this report the authors add to the literature the fact that this variant of an increasingly understood but heterogeneous tumor can occur, and therefore should be considered in the differential of clinically similar tumors.

Secondary Glioblastoma Multiforme in a Child with Disseminated Juvenile Pilocytic Astrocytoma

Secondary glioblastoma multiforme (sGBM) can occur after a long latency period following radiation treatment of various diseases including brain tumors, leukemia, and more benign disorders like tinea capitis. Outcomes of radiation-induced sGBM remain poor in both children and adults. We report a case of a 16-year-old girl with a history of disseminated juvenile pilocytic astrocytoma treated with chemotherapy and craniospinal radiation 9 years prior who developed sGBM in the absence of a tumor predisposition syndrome. She presented with a several-week history of headaches and no acute findings on computed tomography compared to baseline neuroimaging 3 months prior. Repeat computed tomography performed just 3 weeks later for worsening headaches revealed a new large posterior fossa tumor where pathology confirmed the diagnosis of sGBM. In spite of maximal surgical resection, reirradiation, and adjuvant chemotherapy, she died 1 year postdiagnosis. Our case highlights the potential late effects of high-dose cranial radiation, how symptomatology may precede neuroimaging findings, and the rapid formation of sGBM that mirrors that of de novo Glioblastoma Multiforme.

Johann Christian Rosenmüller (1771-1820): A Historical Perspective on the Man behind the Fossa

Introduction The fossa of Rosenmüller, also known as the lateral pharyngeal recess, is a well-established site of origin of nasopharyngeal carcinoma. It is located in the lateral pharyngeal wall behind the cartilaginous portion of the Eustachian tube, the torus tubarius, and is named after Johann Christian Rosenmüller (JCR). Objective We present a history on the life and extensive works of Johann Christian Rosenmüller, a German physician and anatomist. Results Johann Christian Rosenmüller was a dedicated anatomist. In addition to identifying the fossa of Rosenmüller, his influence extends to various other anatomic subjects, including the Rosenmüller gland, the palpebral portion of the lacrimal gland, and the organ of Rosenmüller (i.e., the caudal remnant of the mesonephric duct). He was also an avid speleologist, studying the composition of caves and their life forms. For his contributions to this field, he had a cave in Germany and an extinct species named after him-Rosenmüllerhöhle and Ursus spelaeus Rosenmüller, respectively. Conclusion The fossa of Rosenmüller plays an important role in the growth and surgical treatment of nasopharyngeal carcinoma. We present a brief glimpse into the life of Johann Christian Rosenmüller, for whom it was named.

Pediatric Spinal Cord Tumors

Pediatric spinal cord tumors comprise a rare but challenging subset of neoplasms with regards to neurosurgical and neuro-oncologic management. Spinal cord tumors may present with more common findings of weakness or back pain in older children or with more subtle findings of early handedness or head tilt in younger children and infants. An understanding of the diverse presentation of pediatric spinal cord tumors, their radiographic features and management strategies is crucial as delayed diagnosis may affect clinical outcome. While the vast majority of pediatric spinal cord tumors are histologically benign, their anatomic location often makes them a challenge to treat, both surgically and medically, as there is no universal consensus of the management of these children. Gross total surgical resection is the mainstay of therapy whenever feasible, however, intramedullary tumors pose a unique set of challenges for the neurosurgeon. Adjuvant chemotherapy plays a lesser role in the management of spinal cord tumors compared to their cerebral counterparts. Radiation therapy is a very important adjuvant therapy in the treatment of spinal cord tumors. However, depending on the age of the child and extent of radiation field, this may carry future risks of scoliosis or future secondary malignancy. There has been a relative lack of progress in non-surgical management of children with spinal cord tumors. The rarity of pediatric spinal cord tumors, together with small tissue sizes obtained during surgery portends a lesser understanding of the biologic mechanism of this disease. In this chapter, we will present the most common pediatric spinal cord tumors, their clinical and neuroradiographic features, as well as tumor biology and treatment strategies.

Isolated Sensorineural Hearing Loss as Initial Presentation of Recurrent Medulloblastoma: Neuroimaging and Audiologic Correlates

Medulloblastoma represents 13 % of all childhood primary brain tumors ages 0–14 years and is the most common malignant tumor of childhood with an incidence of 0.51 per 100,000 person per year [1]. Children diagnosed with average-risk medulloblastoma have been reported by several groups to have 5-year survivals greater than 85 % following maximum surgical resection, craniospinal radiation, and adjuvant chemotherapy [2, 3]. patterns of medulloblastoma recurrence may be both local as well as distant and are frequently discovered by worsening symptomatology or by findings on routine neuroimaging. We report a case of a young adult with multiple recurrent medulloblastoma presenting with isolated right-sided sensorineural hearing loss confirmed by audiometry as the only clinical feature of disease recurrence. Magnetic resonance imaging (MrI) revealed restricted diffusion of the cranial nerve VIII (CnVIII) complex that correlated with contrast-enhancing metastatic disease. This unique pattern of medulloblastoma recurrence is important to recognize and highlights the importance of diffusion-weighted imaging (DWI) in the routine disease surveillance of patients diagnosed with medulloblastoma.

Spinal Arteriovenous Lesions

Spinal arteriovenous lesions represent a heterogeneous set of pathologic entities. As our general understanding of these lesions has evolved, so have the classification systems clini‐ cians use to describe them. Historically, the most common classification of arteriovenous fistulas (AVF) has been the Type I-IV classification. More recently these lesions have been described from an anatomical and physiologic perspective.