1/183. bile duct hamartomas (von Meyenburg complexes): value of MR imaging in diagnosis.We present a case of a patient initially believed to have multiple liver metastases based on ultrasound and computed tomography findings in whom a diagnosis of multiple bile duct hamartomas (von Meyenburg complexes) was indicated after ultrasound-guided liver biopsy. magnetic resonance imaging aided greatly in confirming the diagnosis of von Meyenburg complexes, thereby avoiding laparotomy and open liver biopsy.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/183. MR imaging of complex tail-gut cysts.Retrorectal-cyst hamartomas (RCH) are rare developmental tail-gut cystic tumours of the retrorectal space, which occasionally undergo malignant transformation. We describe the magnetic resonance imaging (MRI) findings in two patients with RCH and in a third patient with unclassified sarcoma arising from a RCH. The RCH were hypointense or hyperintense on T1-weighted images and hyperintense on T2-weighted images; they did not enhance and they contained multiple septations. A solid component in the periphery of one cyst was markedly hypointense on T2-weighted images in keeping with fibrous material. The sarcoma arising from the wall of the RCH enhanced and was of intermediate signal intensity on all sequences. MR may help establish the diagnosis of RCH if an unenhanced cystic tumour is discovered in the retrorectal space and it can help detect those rare cases of malignant transformation of these developmental tumours.- - - - - - - - - - ranking = 0.66666666666667keywords = complex (Clic here for more details about this article) |
3/183. Oral-facial-digital syndrome with hypothalamic hamartoma, postaxial ray hypoplasia of the limbs, and vagino-cystic communication: a new variant?We report on a 20-month-old girl with hypothalamic hamartoma, left cerebral atrophy, tongue nodules, oral frenula, micrognathia, hypoplasia of the left ulna, the fibulae, and right tibia, polysyndactyly of the hands and feet, vagino-cystic drainage with hydrometrocolpos, megaloureters, and hydronephrosis, agenesis of urethra, complex partial seizures, and central precocious puberty. The differential diagnosis is discussed. We conclude that the malformation complex in this girl is an oral-facial-digital syndrome, but is different from any of the 11 known subtypes.- - - - - - - - - - ranking = 0.33333333333333keywords = complex (Clic here for more details about this article) |
4/183. Ictal bradycardia in a patient with a hypothalamic hamartoma: a stereo-EEG study.Little is known about bradycardia and cardiac asystole which occur during partial epileptic seizures, especially whether they relate to ictal involvement of well-defined cortical areas. Several reports based on simultaneous electrocardiographic and intracranial depth electroencephalographic monitoring have shown that either the fronto-orbital cortex or the amygdalohippocampal complex could be responsible for such cardiac variations. We performed stereo-EEG recordings in a patient with refractory localization-related epilepsy associated with a hypothalamic hamartoma. We found that other cortical areas, such as the frontocentral region and the temporal neocortex, can contribute to the genesis of ictal bradyarrhythmia. Second, the lesion per se, although located within the hypothalamus, is not involved with this phenomenon.- - - - - - - - - - ranking = 0.16666666666667keywords = complex (Clic here for more details about this article) |
5/183. Nasal chondromesenchymal hamartoma of infancy: the first Japanese case report.The first Japanese case of nasal chondromesenchymal hamartoma, a rare infantile nasal neoplasm, is presented. A 4-month-old Japanese boy was referred to our Centre because of intranasal mass and oculomotor disturbance. Radiological images showed the intranasal tumor extending to the paranasal sinus, orbit, and anterior frontal fossa. Subtotal resection and radiation therapy to residual tumor were performed. There has been no recurrence of the tumor for 13 years. Histologically, the lesion demonstrated admixture of various mesenchymal elements, including cellular spindle cell stroma with occasional myxoid change, nodules of mature/immature cartilaginous tissue, focal osteoclast-like giant cells, and erythrocyte-filled spaces resembling aneurysmal bone cyst. The histology was consistent with the findings presented by McDermott et al. 1986. Immunohistochemically, the spindle cells were positive for vimentin and smooth muscle actin. chondrocytes in the mature cartilaginous tissue were positive for S-100 and vimentin; chondrocytes in the immature cartilaginous tissue were positive for S-100, vimentin, and smooth muscle actin. Ultrastructurally, the spindle cells showed features of either fibroblast or myofibroblast.- - - - - - - - - - ranking = 0.87121025276863keywords = neoplasm (Clic here for more details about this article) |
6/183. A case of familial angiolipomatosis with Lisch nodules.Familial angiolipomatosis is a rare syndrome that may be confused clinically with neurofibromatosis type 1. This condition is most often inherited in an autosomal recessive manner; however, several reports have been published suggesting an autosomal dominant mode of inheritance. Angiolipomatosis, although somewhat disfiguring, is a benign condition with no known association with malignant neoplasms. This is in contradistinction to neurofibromatosis, an autosomal dominant syndrome associated with a myriad of benign and malignant neoplasms. It is, therefore, important to discriminate this entity from neurofibromatosis when a patient presents with multiple subcutaneous tumors and a family history of similar lesions. Described is a case of a prison inmate with a history of seizures and "neurofibromatosis" without clinical documentation. Lisch nodules were noted on the irides. Postmortem examination showed multiple subcutaneous yellow tumors on the chest and arms. Fine-needle aspiration of 1 mass yielded adipose tissue with prominent vessels; histologic sections of another mass showed angiolipoma. The remainder of the autopsy showed significant coronary artery disease and a remote cerebral infarction of the temporal lobe but no signs of neurofibromatosis. We feel that the presence of multiple angiolipomas in combination with Lisch nodules lends credence to the proposed relationship between fatty tumors and neurofibromatosis suggested by other authors.- - - - - - - - - - ranking = 1.7424205055373keywords = neoplasm (Clic here for more details about this article) |
7/183. Spiradenoma arising in a nevus sebaceus of Jadassohn: case report and literature review.nevus sebaceus (NS) of Jadassohn is usually a verrucous plaque on the scalp or face that arises secondary to disordered development of epithelial, pilar, sebaceous, and apocrine structures. The emergence of neoplasia is a late stage in the natural history of NS. Although most neoplastic proliferations are benign, several malignant tumors have arisen in this lesion. We describe the first case of a benign spiradenoma arising in an NS on the scalp in a 72-year-old Caucasian woman. Reexcision was recommended to prevent the development of a second neoplastic process and to avoid the rare occurrence of a malignant transformation of the existing neoplasia. The patient declined reexcision and remains under observation. The spectrum of tumors arising in NS are described and are categorized according to behavior. Syringocystadenoma papilliferum is the most commonly observed benign growth, whereas basal cell carcinoma is the most frequently seen malignant process. The signs of tumor development (benign or malignant) within an NS are reviewed, and treatment recommendations are provided. The clinical course of rare and unique aggressive neoplasms originating in NS is summarized.- - - - - - - - - - ranking = 0.87121025276863keywords = neoplasm (Clic here for more details about this article) |
8/183. Trichoblastoma and sebaceoma in nevus sebaceus.We report two cases of an unusual combined adnexal neoplasm arising in a nevus sebaceus (NS). Clinically, both neoplasms presented in two women (46 and 78 years) as single, partially ulcerated nodules within NS situated on the scalp. Histopathologically, each neoplasm showed distinctive aggregations of basaloid cells with features of trichoblastoma adjacent to aggregations of neoplastic cells exhibiting features of sebaceoma. In both cases, typical features of NS were present. To the best of our knowledge, this unusual combined adnexal neoplasm comprised of trichoblastoma and sebaceoma could not be assigned to any previously described histopathologic entity. This "complex" adnexal neoplasm should be distinguished histopathologically from basal cell carcinoma with sebaceous differentiation and trichoblastoma with sebaceous differentiation.- - - - - - - - - - ranking = 4.5227179305098keywords = neoplasm, complex (Clic here for more details about this article) |
9/183. Cowden's disease (multiple hamartoma syndrome). Report of a limited mucocutaneous form.A case of Cowden's disease has been presented and management considerations documented. Mucocutaneous lesions were the dominant feature of the disease. A rationale for differentiating Cowden's disease from other complex syndromes is considered vital to clinical practice.- - - - - - - - - - ranking = 0.16666666666667keywords = complex (Clic here for more details about this article) |
10/183. Hamartomas of the iris and ciliary epithelium in tuberous sclerosis complex.Astrocytic hamartomas of the retina are the principal ocular manifestation of tuberous sclerosis complex. iris abnormalities are rare in tuberous sclerosis complex and include focal areas of stromal depigmentation and atypical colobomata. We describe 2 patients who were found on histopathological examination to have lesions consistent with hamartomas of the iris pigment epithelium and ciliary body epithelium. iris abnormalities, including pupillary irregularities, were noted on clinical examination prior to the development of iris neovascularization in both patients. These observations suggest that iris abnormalities, including atypical colobomas, may be caused by hamartomas of the iris pigment epithelium and ciliary epithelium in some patients with tuberous sclerosis complex. To our knowledge, hamartomas of tissues derived from the anterior part of the neuroectodermal optic cup have not been reported in cases of tuberous sclerosis complex.- - - - - - - - - - ranking = 1.3333333333333keywords = complex (Clic here for more details about this article) |
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