Fatty filum with different histological features. Case report.

Split cord malformation and fatty filum are completely different clinical entities and thought to arise via different pathophysiologies. Recognition of these distinct lesions in the same patient is important for appropriate diagnosis and management. A 3 year-old boy presented with skin lesions and mild leg weakness suggestive of spinal malformation. Magnetic resonance imaging revealed type II split cord malformation at T12-L1, syringomyelia at T8-T10 levels associated with tethered cord and fatty filum terminale. The patient underwent a T12-L1 laminotomy for the removal of fibrous band between the 2 hemicords and L4-L5 laminotomy for transection of the fatty filum. Histopathological examination of the filum confirmed the presence of bone, fat, and ciliated epithelial cells associated with meningothelial proliferation in the same specimen. We report an unusual case of type II split cord malformation coexisting with a fatty filum which have different histological patterns. To the best of our knowledge, this histological appearance of a fatty filum has not yet been reported and this raises the question of a possible associative or causative relationship between these distinct pathologies.

Fatty filum with different histological features. Case report

Split cord malformation and fatty filum are completely different clinical entities and thought to arise via different pathophysiologies. Recognition of these distinct lesions in the same patient is important for appropriate diagnosis and management. A 3 year-old boy presented with skin lesions and mild leg weakness suggestive of spinal malformation. Magnetic resonance imaging revealed type II split cord malformation at T12-L1, syringomyelia at T8-T10 levels associated with tethered cord and fatty filum terminale. The patient underwent a T12-L1 laminotomy for the removal of fibrous band between the 2 hemicords and L4-L5 laminotomy for transection of the fatty filum. Histopathological examination of the filum confirmed the presence of bone, fat, and ciliated epithelial cells associated with meningothelial proliferation in the same specimen. We report an unusual case of type II split cord malformation coexisting with a fatty filum which have different histological patterns. To the best of our knowledge, this histological appearance of a fatty filum has not yet been reported and this raises the question of a possible associative or causative relationship between these distinct pathologies. (AU)

La diastematomielia y el filum lipomatoso son entidades clínicas completamente diferentes y cuya patogenia se considera diferente. El reconocimiento de estas lesiones en el mismo paciente es importante para el diagnóstico y manejo apropiados. Un niño de 3 años de edad se presentó con marcas cutáneas y moderada debilidad en piernas sugestivos de malformación espinal. La resonancia magnética reveló duplicación de la médula de tipo II y siringomielia T12-L1, que se asoció con anclaje medular y filum terminale lipomatoso. El paciente fue sometido a una laminotomía T12-L1 para la eliminación de la banda fibrosa entre las dos hemimédulas y laminotomía L4-L5 con sección del filum lipomatoso. El estudio anatomopatológico del filum confirmó la presencia de hueso, grasa, y células epiteliales ciliadas, que se asociaron con proliferación meningotelial. Informamos un caso excepcional de diastematomielia tipo II que coexiste con un filum lipomatoso con patrón histológico diferente. En nuestra opinion, este patrón histológico correspondiente al filum lipomatoso no ha sido documentado previamente, y ello suscita cuestiones sobre una posible relación, sea de asociación o cusal, entre estas dos patologías (AU)

Anti-apoptotic and growth-promoting markers in adult medulloblastomas.

AIM: the aim of this study was to investigate the pathologic features, proliferation potential and expression of some anti-apoptotic and growth-promoting markers in adult medulloblastomas. METHOD: we analyzed the immunohistochemical expression of survivin, c-KIT, Bcl-2, fascin, p-53 and Ki-67 in 18 adult medulloblastomas (> 16 years of age). RESULTS: study included 14 males and 4 females (mean age: 22.9 ± 8.2). 14 cases were classical, 2 desmoplastic/nodular and 2 large cell medulloblastomas. Moderate-to-high nuclear survivin expression was observed with high percentages (55 - 100%) in all medulloblastomas while Bcl-2 was mildly positive in only 1 case. Interestingly, mild-to-moderate cytoplasmic c-KIT expression was demonstrated in 16 cases (89%) without membranous accentuation. Fascin expression was observed in 13 medulloblastomas (72%), 9 of which showing moderate to high immunoreactivity. Mild p53 expression was present in only 4 cases (22%). Mean Ki-67 index was 20.6% (range 8 - 55%). CONCLUSION: frequent nuclear survivin expression implies the predominance of anti-apoptotic factors in pathogenesis of adult medulloblastomas. It may also be a potential therapeutic target for adult medulloblastomas. Although Blc-2 immunoreactivity was previously reported in approximately 30% in medulloblastomas, we have observed that it is rarely expressed in the present series of adult medulloblastomas. To our knowledge, this is the first study evaluating fascin expression in medulloblastomas. Its presence may be related to the neuronal differentiation. Mild-to-moderate cytoplasmic c-KIT immunoreactivity without membranous staining in adult medulloblastomas may support the previous studies reporting low level of c-KIT protein expression with lack of activating mutations in medulloblastomas. It seems p53 is rarely involved in the course of develepment of adult medulloblastomas.

Distant muscular (rectus femoris) metastasis from laryngeal squamous cell carcinoma.

OBJECTIVE: We report an extremely rare case of distant metastasis to the rectus femoris muscle from a laryngeal squamous cell carcinoma. METHOD: We present a case report and a review of the world literature concerning distant skeletal muscle metastasis from head and neck squamous cell carcinoma. RESULTS: The patient showed no evidence of distant muscular recurrence at follow up after 21 months; however, two years later he died from brain and suprarenal metastases. CONCLUSION: This report describes the first case of haematogenous distant metastasis to the left rectus femoris muscle from a laryngeal squamous cell carcinoma, without evidence of cervical lymph node metastases. To the best of our knowledge, this is the second reported case of muscular distant metastasis without cervical lymph node involvement. Although the prognosis associated with skeletal muscle metastasis is considered to be poor, surgical excision may be indicated for an isolated muscular metastasis.

Simultaneously occurring vestibular schwannoma and meningioma in the cerebellopontine angle: case report and literature review.

Simultaneously occurring multiple primary brain tumors of different histological types are rare, and the coexistence of schwannoma and meningioma in the same cerebellopontine angle (CPA) without neurofibromatosis is extremely rare. A 57-year-old female patient presented with headache, speech disturbance, left facial numbness and deafness in the left ear. Magnetic resonance imaging demonstrated two different tumors in the left CPA. These tumors were not in continuity. The tumors were totally removed through the left suboccipital approach. Histopathological examination revealed that the large tumor was a vestibular schwannoma and the smaller was a meningioma. Neurofibromatosis was not diagnosed in the patient. No recurrence was observed at the end of 9 years after the operation. The simultaneous occurrence of vestibular schwannoma and meningioma in the CPA appears coincidental. This association must be kept in mind if two different tumors are detected radiologically in the same CPA.

Cyclooxygenase-2 (cox-2) expression and angiogenesis in intracranial ependymomas.

AIM: Cyclooxygenase-2 (Cox-2), the inducible key enzyme in the biosynthesis of prostaglandins, appears to play a role in the regulation of progression, invasiveness and angiogenesis of various neoplasms including some glial tumors. Little is known about the role of Cox-2 in angiogenesis and proliferation of ependymomas. We studied Cox-2 expression, Ki-67 labeling index (Ki-67 LI) and microvessel density (MVD) in 30 intracranial ependymomas and analyzed the relationship among these parameters to evaluate their importance in the tumor biology of ependymomas. RESULTS: The mean Ki-67 LI for all tumors ranged from 1 - 50% (mean 9%). Statistically significant difference was present for Ki-67 LI between ependymomas (grade II, WHO) and anaplastic ependymomas (grade III, WHO) (p < 0.001) (mean Ki-67 LI for ependymoma, 2.8%, for anaplastic ependymomas, 15.6%). Anaplastic ependymomas did not demonstrate a greater vascularization than ependymomas, and the MVD values were 84.5 +/- 39.7 for ependymomas, and 90.6 +/- 61.4 for anaplastic ependymomas. Cox-2 immunohistochemical expression was observed in 19 tumors (63%). Although Cox-2 expression was slightly higher in anaplastic ependymomas, it was not statistically significant. No correlation was found between Cox-2 expression and MVD and Ki-67 LI. CONCLUSION: Similar to morphologic and prognostic heterogeneity in ependymomas, Cox-2 expression, MVD and Ki-67 LI also show a great variability. Other factors may be more important for the proliferation and angiogenesis of ependymomas.

Isolated intracranial Rosai-Dorfman disease mimicking meningioma.

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease (RDD), is an idiopathic histiocytic proliferation affecting lymph nodes. It is typically characterized by painless cervical lymphadenopathy, fever and weight loss. Although extranodal involvement has been reported in diverse sites, intracranial presentation, particularly in the absence of nodal disease is uncommon. To the best of our knowledge, 48 patients with intracranial masses have been reported previously. A 31-year-old man was admitted to our clinic with a 4-month history of progressive headache. His medical history was unremarkable except for occasional fever. There were not any neurological deficit and weight loss. No lymphadenopathy (particularly bilateral cervical) and extranodal involvement in diverse sites were revealed by physical and radiological examinations. Routine hematological and biochemical studies were normal except for mild leukocytosis and elevated erythrocyte sedimentation rate. The patient underwent magnetic resonance imaging (MRI) testing that revealed an enhancing mass in the left temporal lobe. Preoperative diagnosis was meningioma. The patient underwent a left frontotemporal craniotomy with complete resection of the mass. Histopathology was compatible with RDD. Extranodal RDD is rarely found intracranially. Prognosis is benign especially in the absence of nodal disease. It is clinically and radiologically difficult to distinguish from meningioma, and histological examination is essential for a definitive diagnosis.

DNA ploidy and nuclear morphometry in adult intracranial ependymomas.

The identification of prognostic parameters in ependymomas remains an important but controversial issue in particular to the significance of histopathological features. Intracranial location is a rare presentation for adult ependymomas. DNA ploidy status and nuclear morphometric features of ependymomas are not well-established. We retrospectively studied the clinicopathologic features of 12 adult intracranial ependymomas and performed image cytometric DNA analysis with nuclear morphometry. The overall survival rate was 75%. Two cases were anaplastic ependymomas. Statistically, histology failed to demonstrate a correlation with clinical outcome or overall survival. DNA ploidy (p = 0.033), DNA index (p = 0.016) and 5c exceeding rate (p = 0.017) were statistically correlated with clinical outcome, but not correlated with overall survival. Nuclear morphometric features measured using 3 different data selection methods were not correlated with clinical outcome or overall survival. It is difficult to identify prognostic factors in a series of cases with limited number. The problem is complicated by the fact that the common criteria used for recognizing malignancy in gliomas are not completely reliable in ependymomas. Nuclear morphometric findings support the general impression that ependymomas are morphologically highly variable tumors. In spite of the small sample size of our series and other factors influencing survival such as the extent of resection and malignant histology, aneuploidy can be suggested as a promising factor, which may reflect potential aggressiveness of the tumor in adult intracranial ependymomas.

Dysembryoplastic neuroepithelial tumor in the left lateral ventricle.

Extracortical location of dysembryoplastic neuroepithelial tumor (DNT) is rare. We present a case of DNT localized in the left lateral ventricle of a 21-year-old female patient. Magnetic resonance imaging of the brain showed a round mass lesion in the floor of the anterior horn of the left lateral ventricle. The lesion was hypointense on T (1)-weighted images and slightly hyperintense on T (2)-weighted images without contrast enhancement. Total removal of the yellowish-white colored soft tumor was performed through a transcallosal approach. Histologically, the tumor was of simple type of DNT that was composed of small oligodendrogliocytes clustered tightly about perivascular spaces with neurons floating in mucoid pools showing an eosinophilic fibrillary matrix in the background. The recognition of DNT with an unusual location in lateral ventricle has therapeutic and prognostic significance since DNT is curable by surgical excision and radiation therapy is of no obvious benefit.

Schwannoma of the lateral ventricle: eight-year follow-up and literature review.

Intracerebral and intramedullary schwannomas are uncommon; but, in general, spinal intramedullary schwannomas are more frequent than intracerebral schwanomas. We present a case of right lateral ventricle schwannoma in a 21-year-old man and review the associated literature. The 21-year-old right-handed man presented with loss of the left-eye vision approximately 8 months before referral to an ophthalmologist. The patient was immediately subjected to computed tomography (CT) scan, which showed an enhanced lesion with cystic component in the right occipital horn of the lateral ventricle. And consecutively, he was admitted to our department. The tumor was evacuated via craniotomy with marked improvement in his clinical state. The postoperative course was uneventful and postoperative CT control showed no residue. On MRI control no recurrence was noted after a follow-up period of 8 years. Intracerebral schwannoma is a rare, benign neoplasm. It is usually located superficially or adjacent to a ventricle. Characteristic imaging features include cyst formation, calcification, and evidence of peritumoral edema or gliosis. The recognition of this benign and potentially curable neoplasm and its differentiation from other neoplasms, some of which have less favourable outcomes, is of obvious importance.

Postoperative radiotherapy for ganglioglioma; report of three cases and review of the literature.

Gangliogliomas are rare tumors of the central nervous system. The definite role of postoperative radiotherapy has not been established. In studying three cases who underwent radiotherapy after subtotal resection, we evaluated the clinical, pathological, radiological features with follow-up of mean 8.1 years. All of three patients were male, and mean age was 22.3 years. All of the cases have temporal lobe lesions and, except for one, they all have seizures. Radiotherapy was given to all patients after surgery. Parallely opposed local fields were used. A total of 54 Gy in 5.5 weeks was given to the mid-plane using a Co-60 tele-therapy device. No recurrence, no new symptoms, and no new neurological deficiency were detected during follow-up.

Intra-hospital use of a telepathology system.

Utilization of telepathology systems to cover distant geographical areas has increased recently. However, the potential usefulness of similar systems for closer distances does not seem to be widely appreciated. In this study, we present data on the use of a simple telepathology system connecting the pathology department and the intra-operative consultation room within the operating theaters of the hospital. Ninety-eight frozen section cases from a past period have been re-evaluated using a real-time setup. Forty-eight of the cases have been re-evaluated in the customary fashion; allowing both ends to communicate and cooperate freely. Fifty of the cases, however, were evaluated by the consultant while the operating room end behaved like a robot; moving the stage of the microscope, changing and focusing the objectives. The deferral rate was lower than the original frozen section evaluations. Overall, the sensitivity was 100%, specificity 98%, negative predictive value 96, 5% and positive predictive value 100%. No significant difference was found for the diagnostic performances between the cooperative and robotic simulation methods.Our results strengthen the belief that telepathology is a valuable tool in offering pathology services to remote areas. The far side of a hospital building can also be a remote area and a low cost system can be helpful for intraoperative consultations. Educational value of such a system is also commendable.

The human tail associated with intraspinal lipoma: case report.

A case of a tail in a 9-month-old baby is reported. Computed tomography and magnetic resonance imaging clearly demonstrated the presence of spina bifida and lipoma continuous from the tail to the spinal canal. A few medical-historical aspects are discussed. The human tail may be related to spinal dysraphism and requires detailed neuroimaging investigation and microsurgery.