Neuroblastoma, ganglioneuroblastoma and ganglioneuroma are derived from primordial neural crest cells and can be conceptualized as three different maturational manifestations of a common neoplasm. To assess the validity of immunohistochemistry and DNA Ploidy in the diagnosis of neuroblastic tumor in terms of prognostication, histologic and immunohistochemical evaluation with NB-84, neuron specific enolase(NSE) and S-100 protein and flow Cytometric DNA analysis were done on 21 neuroblastomas and 19 ganglioneuromas. Thirteen of 21 neuroblastomas were undifferentiated and 8 differentiating in type. Eleven of the 19 ganglioneuromas were mature in type and 8 had immature foci. Eighty one percent of neuroblastomas were positive for NB-84, 100% for NSE and 67% for S-100 protein, respectively. All ganglioneuromas were positive for NSE and S-100 protein, in contrast, only immature foci in ganglioneuroma were positive for NB-84. B-84 reacted positively with undifferentiated and differentiating neuroblasts including neuropil but not with mature ganglion cells. In contrast, NSE reacted positively with all components of neuroblastic tumor and S-100 protein mainly with cells of Schwannian differentiation. Three of eight(37.5%) differentiating neuroblastomas were strongly positive for NB-84 in contrast with seven of thirteen(53.8%) undifferentiated tumors, reflecting that undifferentiated cells tended to be positive for NB-84 in neuroblastoma. Twenty two percent of neuroblastoma showed diploidy and 78% aneuploidy including 11% of near-diploidy. Seven of eight(87.5%) differentiating neuroblastomas in contrast with seven of ten(70%) undifferentiated tumors showed aneuploidy. By contrast, 53% of ganglioneuroma showed diploidy and 47% aneuploidy with DNA index ranged from 1.12 to 1.19. Three of nine(33.3%) mature ganglioneuromas in contrast with five of eight(62.5%) ganglioneuromas with immature foci showed aneupolidy. Differentiating neuroblastoma tended to be aneuploid and ganglioneuroma with immature foci tended to be near-diploid. In conclusion, immunohistochemistry for NB-84, NSE and S-100 protein is useful for confirming neuronal, both neuronal and Schwannian, and Schwannian differentiation, respectively. Immunohistochemistry together with flow cytometric DNA analysis would be helpful to confirm the immature foci in ganglioneuroma.
Neuroblastoma

Peri-vater divertieula may interfere with the normal flow of biliary and pancreatic secretion, thus leading to pancreatobiliary diseasa and the very presence of juxtapapillary diverticulum makes cannulation technically difficult and contributes to the low success rate. In this present series, the relationship between duodenal diverticula and age, sex, and biliary-pancreatic pathology was stud.ied in 1400 patients examined with ERCP. The results are as follows. 1) Duodenal diverticula was demonstrated in 73 of 1400 patients(5, 2%). 2) The incidence of duodenal divertieular increased with age and they were more frequent in men. 3) The cannulation of one or both duct system was suecessful in 69 of the 73 patients, the success rate was 94. 5%. 4) The biliary pathology was found in 41 of the 73 patients and the pancreatic pathology in 6 of the 73 patients. The rnost common finding was biliary calculi which were demonstrated in 35 patients(47. 9%).
Catheterization ; Cholangiopancreatography, Endoscopic Retrograde* ; Diagnosis* ; Diverticulum* ; Gallstones ; Humans ; Incidence ; Male ; Pathology

Hodgkin's disease, as a poly-morphous group of lymphoma, is a disease of reticuloendothelial system, skin and usually affects the lymph node and so primarily, predominantly by proliferation of lymphocyte and histiocyte. This patient is a 42 years old Housewife who was suffered from severe itching sensation on the entire body since she had illed with exfoliative decmatitis, 7 years ago. Skin lesion showed brownish colored hyperpigmentation on entire body, scattered or grouped lichenified patch with scale on extremities and hypertrophy on the palms and soles. On physical examination, she had a egg-sized, right cervical lymph node swelling and chestnut sized, left cervical lymph node swelling without pain. On laboratory findings showed within normal limits. Sternal bone marrow aspiration revealed nonspecifie findings. A chest X-Ray revealed mass shadow on right upper mediastinum. Histologically finding of the skin revealed nonspecific dermatitis picture. On diagnostic excisional biopsy of left cervical lymph node showed numerous Hodgkin's cell Liver scanning didn't show space occeuying lesion. Above as a result, by clinical manifestation, X-ray findings and histological findings of cervical lymph-node, the diagnosis of Hodgkins disease with nonspecific dermatitis was confirmed. Literature was briefly reviewed for the discussion of clinical and histological findings of this dlsease.
Adult ; Biopsy ; Bone Marrow ; Dermatitis* ; Diagnosis ; Extremities ; Histiocytes ; Hodgkin Disease* ; Humans ; Hyperpigmentation ; Hypertrophy ; Liver ; Lymph Nodes ; Lymphocytes ; Lymphoma ; Mediastinum ; Mononuclear Phagocyte System ; Physical Examination ; Pruritus ; Sensation ; Skin ; Thorax

We report three cases of neuroendocrine tumors of the lung characterized by large pleomorphic cell with frequent mitosis, which show neuroendocrine differentiation by both light microscopy or electron microscopy and iminunohistochemistry. These tumors have been categorized as large cell neuroendocrine carcinoma by Travis et al.(1991) in contrast with non-small cell lung cancer with neuroendocrine differentiation. In the latter, neuroendocrine differentiation is not evident by light microscopy and must be demonstrated by imunohistochemstry or by electron microscopy. The prognosis of large cell neuroendocrine carcinoma, together with non-small cell lung cancer with neuroendocrine differentiation, appears to be worse than cancer without neuroendocrine differentiation and intermediate between atypical carcinoid and small cell lung cancer. Larger numbers of patients will be needed to demonstrate significant differences in survival.
Lung Neoplasms

Neurocutaneous melanosis is a very rare congenital syndrome characterized by the presence of large or multiple congenital melanocytic nevi and benign or malignant melanotic tumors of the central nervous system. We report herein a case of neurocutaneous melanosis with leptomeningeal melanosis and a malignant melanoma of the right temporal lobe in a 46-year old man. The case is exceptional as regards the late onset of symptoms and death. Even without a malignant melanoma, the symptomatic neurocutaneous melanosis has a fatal course. The syndrome is rare but lethal, so the dermatologist should be aware of this syndrome when evaluating the patients with large or numerous congenital melanocytic nevi so as to watch for the usual signs of increased intracranial pressure and to take prompt palliative measures.
Central Nervous System ; Humans ; Intracranial Pressure ; Melanoma ; Melanosis* ; Nevus, Pigmented ; Temporal Lobe

Pigmented(melanotic) schwannoma is a very rare variant of schwannoma that characteristically has massive cytoplasmic melanin. Since it was described in 1946 by Bjorneboe, about 44 cases have been reported in the English literature. It has a relatively benign clinical course, but the cases arising in the cranial nerve and sympathetic chain show aggressive behavior with malignant potential. We herein report a typical case of pigmented schwannoma with light microscopic findings. The results of immunohistochemical and electronmicroscopic study are also presented. The patient was a 30 year-old Korean male who had a mass in his posterior neck for 10 years that recently.began to induce neurologic manifestations. The tumor, which was mainly in the extramedullary intradural space of the cervical canal extending to the extradural space and soft tissues of the neck, was relatively well defined and composed of black solid tissue. Microscopic, densely pigmented spindle cells forming fascicles, nuclear palisading, whorling and polygonal cells with vacuolated or clear cytoplasm were characteristic. Immunohistochemically, the tumor cells were positive for vimentin, S-100 protein, and HMB-45. Electron microscopic study revealed that the tumor cells have interdigitating cytoplasmic processes containing varying stages of melanosomes with a very focal basal lamina and Luse body like collagen bundles.
Male ; Humans

Biliary cystadenoma of the liver is a rare multilocular cystic neoplasm of biliary origin. it occurs most often in middle aged women and rarely in children. Histogenesis of this tumor is uncertain. It may be developmental in origin arising from aberrant hamartomatous bile ducts or ectopic rests of embryonic biliary cystadenoma of the liver discovered at 8 month of intrauterine fetal life. This case supports its congenital theory.
Child ; Male ; Female ; Humans

A case of congenital rhabdomyoma of the heart in a 5-month-old Korean infant is described. The patient presented with a congenital supraventricular tachyarrhymia that was detected in utero by fetal sonography. The tumor was multiple, but no obvious association with tuberous sclerosis complex was demonstrated. Microscopic examination revealed classic "spider cells" with rich glycogen content. Ultrastructurally, the cells contained numerous leptofibrils, clumped Z band material, and desmosome-like cell junctions. The case is a second documented case of cardiac rhabdomyoma in this country, and its presentation as an etiological factor of supraventricular tachycardia is a very unusual manifestation.
Female ; Heart Neoplasms/complications/*congenital/ultrastructure ; Humans ; Infant ; Rhabdomyosarcoma/complications/*congenital/ultrastructure ; Tachycardia, Supraventricular/*congenital/etiology

BACKGROUND: Clonidine, an alpha2 adrenoceptor agonist, has been known to have an antiallodynic effect in many animal and human studies. Clonidine, however, acts on imidazoline receptors as well as alpha2 adrenoceptors. Recently, the effect of clonidine on the symapthetic nervous system was reported to be mediated via the activation of the imidazoline receptor system but not the alpha2 adrenergic receptor system. Therefore, we conducted a behavioral test to investigate the effects of alpha2 adrenoceptors and imidazoline receptors on mechanical allodynia in rats with spinal nerve ligation (SNL) injury. METHODS: Male Sprague Dawley rats were prepared with tight ligation of the left lumbar 5th and 6th spinal nerves and chronic lumbar intrathecal catheter implantation for drug administration. Using a von Frey hair (VFH) test, we examined the effects of intrathecal (IT) brimonidine (0.03 - 3 microgram), clonidine (3 - 10 microgram), and rilmenidine (1 - 30 microgram) in SNL rats. Measurements of the baseline value VFH test was conducted at each dose to compare with the preoperative state. In addition, an antagonistic study with rauwolscine or yohimbine was performed to investigate the reversal of antiallodynic effects of each agonist. Allodynic thresholds for the withdrawal response of the left lesioned hindpaw to VFH stimuli were assessed and converted to %MPE. RESULTS: The antiallodynic effects of brimonidine, clonidine, and rilmenidine were produced in a dose dependent manner. The antiallodynic effects of IT brimonidine but not rilmenidine were significantly antagonized by alpha2 antgonists rauwolscine and yohimbine (P < 0.05). CONCLUSIONS: The results suggest that mechanical allodynia produced by a SNL injury is reduced by an imidazoline receptor agonist as well as alpha2 adrenergic receptor agonists and sympathetic activation is more likely mediated by spinal imidazoline receptors.
Adrenergic Agonists ; Animals ; Catheters ; Clonidine ; Hair ; Humans ; Hyperalgesia* ; Imidazoline Receptors* ; Ligation* ; Male ; Nervous System ; Rats* ; Rats, Sprague-Dawley ; Receptors, Adrenergic* ; Spinal Nerves ; Yohimbine ; Brimonidine Tartrate

A case of aneurysmal ("angiomatoid") fibrous histiocytoma (AFH) in a 12-year-old girl is presented with its unusual clinicopathologic features. The lesion had the full microscopic characteristics of AFH described in prior reports, but it also had some features that differed from the original description of the disorder, such as the involvement of subcutis, its occurrence in the scalp, and a documented history of minor trauma. The lesion clinically resembled the gross features of hemangioma. The experience in the present case raises the need for considering AFH as one of major differential diagnosis of nodular cutaneous tumors in children that simulate malignancy such as angiomatoid malignant fibrous histiocytoma and Kaposi's sarcoma.
Child ; Diagnosis, Differential ; Female ; Hemangioma/pathology ; Histiocytoma, Benign Fibrous/*pathology ; Humans ; Skin Neoplasms/diagnosis/*pathology