1/37. A case of juxtaglomerular cell tumor diagnosed preoperatively.A case of juxtaglomerular cell tumor, the fourth case in the world diagnosed preoperatively, was reported. Neither strict sodium restriction nor loading of upright position increased a grade of hyperreninemia observed at recumbant position on ordinary diet, but Pald elevated in response to them. Abnormal glucose tolerance was considered to be due to hypopotassemia. Selective renal arteriography revealed a small number of tumor vessels, a lucent area on the nephrogram and early appearance of the renal vein of the diseased side. Histologically, the tumor contained some canalicular structures among the polygonal nonepithelial tumor cells with rhomboid bodies. These findings suggested that this tumor was a hamartoma originated from the juxtaglomerular apparatus.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
2/37. spinal cord hamartoma: case report.OBJECTIVE AND IMPORTANCE: spinal cord hamartomas are infrequently mentioned in the literature. The authors present a unique report detailing the clinical presentation of a spinal cord hamartoma, with supporting radiographic and pathological data. CLINICAL PRESENTATION: A 26-year-old man presented with progressive right upper extremity weakness. Imaging studies revealed an exophytic cervical spinal cord mass. INTERVENTION: Open biopsy was undertaken and revealed tethering of the lesion to the dura. A pathological examination revealed a spinal cord hamartoma. CONCLUSION: The patient's symptoms improved postoperatively, suggesting that tethering of the spinal cord was responsible for the symptoms. Although unusual, hamartoma should be included in the differential diagnosis of an exophytic spinal cord lesion.- - - - - - - - - - ranking = 4.3867739147954keywords = spinal (Clic here for more details about this article) |
3/37. Malignant glial tumor arising from the site of a previous hamartoma/ganglioglioma: coincidence or malignant transformation?Gangliogliomas are generally considered benign tumors. Although more commonly found in the brain, spinal cord ganglioglioma is a well established, albeit infrequent, entity. We describe a 2-decade clinical course of a patient initially diagnosed with a thoracolumbar 'glial-neuronal hamartoma' at age 4. Seventeen years after his first operation, local recurrence was noted. Despite subsequent multiple gross total resections and adjuvant therapy, histologic features became increasingly ominous and ultimately proved fatal. This is an unusual report and histologic presentation of a resected spinal cord ganglioglioma recurring as an anaplastic ependymoma/astrocytoma and subsequently a glioblastoma. It is quite likely that the originally resected ganglioglioma was actually part of a primitive neuroectodermal tumor which had undergone extensive maturation.- - - - - - - - - - ranking = 1.7547095659182keywords = spinal (Clic here for more details about this article) |
4/37. Midline spinal cord hamartomas: MR imaging features of two patients.Congenital midline spinal hamartomas are relatively rare. patients harboring this anomaly are generally asymptomatic, but present with an overlying skin anomaly. MR imaging depicts a mass that is isointense with the spinal cord on all sequences, and may show a dermal sinus tract that tethers the cord at the level of the lesion. We report the MR features of congenital midline spinal hamartoma in two children.- - - - - - - - - - ranking = 6.1414834807136keywords = spinal (Clic here for more details about this article) |
5/37. Neurocutaneous vascular hamartomas mimicking Cobb syndrome. Case report.The authors report the rare case of a patient with neurocutaneous vascular hamartomas mimicking Cobb syndrome. An 8-year-old boy was admitted to the authors' hospital with progressive urinary disturbance and upper back pain. Multiple skin nevi had been noted at the child's birth. Radiological examination revealed multiple cavernous angiomas in the spinal cord in the same metamere in which the skin nevi had been observed and also in the left cerebral hemisphere. His symptoms gradually improved without surgical intervention. Four years later he was readmitted because of a cerebral hemorrhage involving the left cerebral peduncle. Nonsurgical treatment was chosen because his symptoms promptly improved. To the best of the authors' knowledge, this is the first case of multiple cavernous angiomas in the brain and spinal cord associated with skin nevi. The authors discuss this clinical entity and the significance of the disease.- - - - - - - - - - ranking = 1.7547095659182keywords = spinal (Clic here for more details about this article) |
6/37. Inner ear malformations and hearing loss in linear nevus sebaceous syndrome.OBJECTIVE: Linear nevus sebaceous syndrome (LNSS) is a rare disorder comprising of nevus sebaceous, seizures, and mental retardation. While extensive literature describe the dermatologic, neurologic, as well as ophthalmologic manifestations of this LNSS, otologic problems have not been previously described. The objective of this report is to describe the otologic manifestation of a patient with LNSS. methods: A child with LNSS was referred to the Otologic clinic for evaluation of hearing loss. Pertinent findings on history, physical findings, audiometric testing, and imaging studies are discussed. RESULTS: Audiometric testing showed bilateral conductive hearing loss. Computerized tomography of the temporal bone demonstrated widened internal auditory canals and dysplastic lateral semicircular canals. CONCLUSION: LNSS can be associated with hearing impairment and inner ear malformations. The evaluation of a child with LNSS should include otologic and audiologic testing.- - - - - - - - - - ranking = 2keywords = canal (Clic here for more details about this article) |
7/37. Hamartomatous endocervical polyp with heterologous mesenchymal tissue.We present an endocervical polyp with heterologous elements. Although a few neoplastic cervical lesions with cartilaginous and adipocytic heterologous tissue have been reported, an endocervical polyp with heterologous cartilage and adipose tissue has not been reported before our case. The patient was a 33-year-old woman who presented with abnormal uterine bleeding. On physical examination, there were no remarkable findings other than a cervical polyp protruding into the cervical canal. The polyp was removed. Pathological examination revealed an endocervical polyp with typical epithelial features. The stroma of the polyp contained mature cartilage islands and adipose tissue. There were also many thick-walled vascular structures. Neither stromal periglandular condensation nor atypia was found. Mitotic figures were not observed. Arteriolar structures did not contain internal elastic lamina. In our opinion, these pathological findings are all consistent with a hamartomatous lesion rather than with a true neoplasm.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
8/37. hamartoma in the internal auditory canal.An 11 year old girl presented with hearing loss in her left ear and left trigeminal and facial nerve palsy. Radiological examinations revealed an enlargement of the left internal acoustic canal and the existence of a mass protruding from the canal into the cerebellopontine angle. The partial resection of the mass by a suboccipital craniectomy resulted in ceasing the progression of her symptoms. Histological diagnosis was hamartoma with cerebellar heterotopia. The concurrence of heterotopic cerebellar tissue could help to understand the pathogenesis of hamartomas.- - - - - - - - - - ranking = 6keywords = canal (Clic here for more details about this article) |
9/37. A rare case of a sebaceous nevus in the external auditory canal.We report the case of a patient who came to us for evaluation of a progressive unilateral hearing loss and who was found to have a sebaceous nevus in an unusual location: the external auditory canal. A sebaceous nevus is a congenital organoid mass that occurs primarily on the face, scalp, and periauricular regions. Despite the predilection of sebaceous nevi for the head and neck, reports of this lesion rarely appear in the otolaryngology literature. Left untreated, the lesion can progress through three stages of gross and histopathologic development; a sebaceous nevus begins as a small benign papule, grows into an enlarging mass with different characteristics, and ultimately becomes a secondary neoplasm. The lesion's potential for malignant transformation and its association with syndromes underscores the importance of prompt recognition and appropriate management. This case report adds the sebaceous nevus to the differential diagnosis of external auditory canal lesions and provides essential information about this rare mass.- - - - - - - - - - ranking = 6keywords = canal (Clic here for more details about this article) |
10/37. Hamartomas of the internal auditory canal: report of two cases.OBJECTIVE AND IMPORTANCE: To highlight the clinical, radiological, and surgical findings and therapeutic options for this rare entity, which may mimic a purely intrameatal vestibular schwannoma, and to define the particular aspects of preoperative differential diagnosis and surgical management. CLINICAL PRESENTATION: Two patients presented with clinical findings typical of vestibular schwannomas, i.e., tinnitus, hearing loss of 30 dB, and an intrameatal contrast-enhancing lesion on magnetic resonance imaging studies. TECHNIQUE: The lesions were exposed via a suboccipital transmeatal approach, and tumor infiltration of the cochlear and/or facial cranial nerves was identified. In view of the unclear intraoperative histology, surgical management was based on criteria of cranial nerve function. In Patient 1, after nerve decompression by subtotal tumor removal, preserved auditory brainstem responses and facial nerve electromyography indicated functional nerve preservation and facilitated the decision for partial resection. In Patient 2, minimal tumor dissection resulted in complete loss of auditory brainstem response without reversibility. Therefore, a radical tumor removal was performed that sacrificed the cochlear but preserved the facial nerve. CONCLUSION: Symptoms and signs of internal auditory canal hamartomas are congruent with other typical pathological lesions of the internal auditory canal and cerebellopontine angle. Accurate preoperative diagnosis by radiological means is not possible, but careful evaluation of the different signal intensities on magnetic resonance imaging studies may indicate this rare pathological condition. Intraoperative surgical findings of tumor infiltration of the faciocochlear cranial nerve complex may support simple observation. In view of the nonneoplastic characteristic of these lesions, a more conservative approach is justified. The decision should be based on the functional status of the cranial nerves, for which reliable electrophysiological monitoring is indispensable.- - - - - - - - - - ranking = 6keywords = canal (Clic here for more details about this article) |
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