1/130. Linear epidermal nevus and nevus sebaceus syndromes: a clinicopathologic study of 3 patients.BACKGROUND: Linear epidermal nevus syndrome and linear sebaceus nevus syndrome are rare neurocutaneous syndromes characterized by epidermal nevi, epilepsy, and mental retardation. Pathologic descriptions of the central nervous system findings in such patients are rare. DESIGN: We examined the clinicopathologic features of 2 patients with linear epidermal nevus syndrome and 1 with nevus sebaceus syndrome who underwent surgical resections for chronic epilepsy in a tertiary referral center with a high volume of epilepsy surgery. RESULTS: patients included 3 females, aged 11 months (patient 1), 8 years (patient 2), and 2 1/2 years (patient 3) at the time of surgery. The duration of seizures prior to surgery was 11 months, 6 years, and 28 months, respectively. Two patients had epidermal nevi involving the head region (patients 1 and 3), and 1 had a nevus sebaceus of Jadassohn (patient 2); patient 2 had a choristoma, and patient 3 had a dermoid cyst in the eye region. Patient 1 demonstrated hemimegalencephaly radiographically. Histologic examination of resected cortical tissue in patients 1 and 2 demonstrated severe diffuse cortical dysplasia characterized by a disorganized cortical architectural pattern, a haphazard orientation of cortical neurons, and increased molecular layer neurons. Gyral fusion was seen in patient 1. Pial glioneuronal hamartomas were observed in patient 1. Prominent cortical astrocytosis was seen in patients 1 and 2, and foci of microcalcification were evident in patient 1. Cortical dysplasia was milder in patient 3 and consisted of an increased number of molecular layer neurons. Neuronal heterotopia was observed in all 3 patients. CONCLUSION: The spectrum of neuronal migration abnormalities in the setting of these syndromes may be variable in terms of its histologic phenotypic manifestations.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/130. 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 = 4.1325555072487keywords = neoplasm (Clic here for more details about this article) |
3/130. 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 = 8.2651110144974keywords = neoplasm (Clic here for more details about this article) |
4/130. 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 = 4.1325555072487keywords = neoplasm (Clic here for more details about this article) |
5/130. oral manifestations of Schimmelpenning syndrome: case report and review of literature.Schimmelpenning syndrome (SS) is characterised by specific skin manifestations, skeletal defects, and central nervous system abnormalities. Here, the SS is briefly reviewed, and the oral and dental manifestations are described in a patient whose medical findings were previously published and included severe hypophosphatemic rickets. Significant oral and dental features included papillomatous lesions of the gingiva, hemihyperplasia (hemihypertrophy) of the tongue, bone cysts, aplasia of teeth, enlarged pulp chambers, hypoplastic or absent enamel, and an odontodysplasia-like permanent tooth.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/130. 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 = 20.662777536243keywords = neoplasm (Clic here for more details about this article) |
7/130. Fine needle aspiration cytology of mesenchymal hamartoma of the liver. A case report.BACKGROUND: Mesenchymal hamartoma (MH) of the liver constitutes the third or fourth most common tumor of the liver in childhood and occurs most commonly in the first two years of life. MHs of the liver are seldom aspirated, and reports on the role of fine needle aspiration (FNA) in the diagnosis of MH are scarce. Clinically, cytologically and even histologically, MH can be mistaken for a number of reactive and neoplastic hepatic lesions that may occur in children under 2 years of age. CASE: A 10-month-old Pakistani female presented with a history of a right-sided, nonpainful abdominal swelling. Abdominal computed tomography showed a large, partly solid and partly cystic, heterogeneous hepatic mass. FNA cytology showed clusters of both epithelial and mesenchymal/spindle-shaped cells with pieces of loose connective tissue. A cytologic differential diagnosis of mesenchymal hepatic hamartoma and hepatoblastoma of the possible mixed mesenchymal/epithelial subtype was rendered. The histopathologic diagnosis of the resected tumor mass was benign mesenchymal hamartoma of the liver. CONCLUSION: In children under 2 years of age who present with partly solid and partly cystic hepatic masses, the possibility of MH of the liver should be considered. FNA has a role in the diagnosis of MH. The cytopathologist should be aware of the patient's age, radiologic features and cytologic appearances of this rare, benign neoplasm. Histologic examination of tru-cut biopsies and immunohistochemical stains can help to exclude other pediatric neoplasms that may show cytologic features similar to or mimicking those of MH.- - - - - - - - - - ranking = 8.2651110144974keywords = neoplasm (Clic here for more details about this article) |
8/130. Segmental neurofibromatosis in association with nevus sebaceus of Jadassohn.We describe an unusual case involving the simultaneous occurrence of segmental neurofibromatosis (Type V NF) in a patient with a large nevus sebaceus of Jadassohn in the same physical distribution. Causative mechanisms of development of these 2 genetic disorders have not been definitively linked. Factors producing these diseases probably involve similar tissues at the same point in development because both have been reported in association with central nervous system anomalies and have been classified among the neurocutaneous syndromes. This is a case of a nevus sebaceus occurring in association with and in the same physical distribution as segmental NF. These disorders most likely represent a spectrum of disease within the phakomatoses.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/130. Two cases of ectopic hamartomatous thymoma.Ectopic hamartomatous thymoma (EHT) is a rare benign neoplasm. Since it was named by Rosai et al. in 1984, 24 cases have been reported. We herein report two cases of EHT, one of which presented with massive myoid cells, and review the literature related to EHT. Both of our cases displayed the typical features of EHT: (1) nests of epithelial cells, including solid, cystic, or glandular epithelial islands; (2) spindle cells dominating the microscopic picture; and (3) adipose cells which intermingle haphazardly to impart a hamartomatous quality to the tumor. In this paper, we observed massive myoid cells and the transition from spindle epithelial cell to myoid cell in one of our cases. Immunohistochemical examinations showed that the main component of EHT, spindle cells, was positive for cytokeratin and epithelial membrane antigen (EMA). Intriguingly, the myoid cells simultaneously expressed cytokeratin, EMA, myoglobin, and creatine kinase-mm, suggesting that myoid cells may originate from epithelial cells and are an intermediate state between epithelial cells and muscular cells.- - - - - - - - - - ranking = 4.1325555072487keywords = neoplasm (Clic here for more details about this article) |
10/130. Clear cell trichoblastoma in association with a nevus sebaceus.nevus sebaceus is a hamartoma that is frequently associated with various neoplasms. Among the neoplasms observed in sebaceus nevi, trichoblastomas are the most common. The present case, to my knowledge, is the first description of a clear cell variant of trichoblastoma.- - - - - - - - - - ranking = 8.2651110144974keywords = neoplasm (Clic here for more details about this article) |
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