Giant congenital melanocytic nevus is found in 0.1% of live born infants. If present, this lesion has a 6.3% chance to develop malignant melanoma. We report such a case in a 22-year-old woman who had multiple pigmented skin lesions since birth. Rapidly growing masses were recently detected in the 19 cm-sized occipital pigmented lesion. Removed scalp lesion revealed yellowish white lobulated soft nodules in the background of pigmented nevus. Microscopically, the nodules consisted of epithelioid cells with prominent nucleoli, and pleomorphic cells including signetring cells. These cells seldom contained melanin pigment. There were metastatic aggregates of tumor cells in the cervical lymph node, which were reminiscent of germinal centers of lymph nodes. S-100 protein immunostaining was helpful to distinguish them. Incidentally, focally scattered pigmented spindle cells were seen in the capsule of a lymph node
Infant ; Male ; Female ; Humans ; Neoplasm Metastasis

The cliniopathological analysis was done on the 104 cases of malignant melanoma diagnosed at the Seoul National University Hospital (SNUH) from 1984 to 1993. The basic clinical data and the pathological items were based on the New Mexico Melanoma Registry Worksheet. The results were as follows. The male to female ratio was 1 : 0.79. Primary cutaneous melanoma was more common in the male (M : F=1 : 0.56) but primary extracutaneous melanoma with slight female dominancy (M : F=1 : 1.25). The peak age was the 6th decade in both cutaneous and extracutaneous malignant melanoma. In 66% (35 cases) of primary cutaneous malignant melanoma, the primary site was located in the acral area (including cases of acral lentiginous and nodular type), of which 63% (41% of total cutaneous melanoma) was acral lentiginous type. Major components of tumor cells were epithelioid. Clark's level of tumor was III or more at the time of the first visit in the majority of the cases (85%). The incidence rate of extracutaneous melanoma was 34.6% (36 cases) among the primary melanoma, and the eyeball (17.3%) was the most prevalent organ. All these features suggest that the racial difference between the Korean and the Caucasian is evident and also that etiologic role of sun damage is not quite marked in the Korean. We also suggest that an early detection program is very important to cure this malignant tumor.
Female ; Humans ; Incidence ; Male ; Melanoma* ; New Mexico ; Seoul ; Solar System

The authors observed one case of typical Kaposi's sarcoma who was 24 year old male. This patient complained of dark-brownish pigmented macules and oozing ulcer on the right knee joint area since at birth and the lesions were slowly growing and increased the number with pain. The skin lesions revealed 3*2.5cm sized dark-brownish colored macuIe, 2.5*3*1cm sized blood discharged nodule and 1* 1cm to l. 5* l. 5cm sized dark-brownish atrophic macules on the right inguinal area.Histopathologically, a biopsy was done on the right knee joint area, the finding showed angiomatous and fibrotic nature, I.e. the blood vessels in the dermis were dilated and increased in number. The endothelial cell was large and protrude into the lumen and showed small group of extravasated erythrocytes in granulation tissue, and showed extensive proliferation of spindle shaped cells that extended irregularly into various direction.
Biopsy ; Blood Vessels ; Dermis ; Endothelial Cells ; Erythrocytes ; Granulation Tissue ; Humans ; Knee Joint ; Male ; Parturition ; Sarcoma, Kaposi* ; Skin ; Ulcer ; Young Adult

In contrast to the nodular renal blastema which is defined by nests of primitive metanephric cells after 36 weeks of gestation, the nephroblastomatosis is characterized by neoplastic proliferation of the primitive cells. This lesion is presumed to be closely related to the development of Wilms' tumor. We report a case of bilateral nephroblastomatosis associated with Wilms' tumor in a child. This 4 1/2 year-old girl was admitted because of a 10 cm-sized round mass in the right kidney, and smaller nodules in the left kidney and the lung. After three cycles of chemotherapy and subsequent disappearance of the nodules in the left kidney and lung, she underwent a right nephrectomy and a wedge resection of the left kidney. A round Wilms' tumor mass was seen in the lower pole of the right kidney. Remaining right renal cortex showed multiple, slightly depressed gray-white nodules associated with multiple samll cysts. They were comprised of multifocal subcapsular nests of primitive nephrogenic cells with focal tubular or glomerular differentiation. They resembled fetal renal tissue. In the left kidney, similar nests of primitive cells were also noted. These lesions were interpreted as multifocal perilobar type of nephroblastomatosis.
Child ; Male ; Female ; Humans

No abstract available.

No abstract available.
Cervix Uteri* ; Female ; Health Expenditures*

The presense of pectus excavatum in Marfan's syndrome may complicate cardiac operation by making midline sternotomy technically more difficult and limiting the operative exposure of the heart. We operated on a 33 year old male patient with Marfan's syndrome and severe pectus excavatum who had severe mitral regurgitation and moderate aortic regurgitation with 52mm aortic root dilation. The operative field was adequately exposed through a midline sternal incision with two sternal retactors. The patient underwent Bentall operation and mitral valve replacement. The repair of pectus excavatum was performed after completion of CPB and the administration of protamin. Permanent internal stabilization achieved by overlapping of the ends of lower ribs and reinforced with sternal closure wire.
Adult ; Aortic Valve Insufficiency ; Funnel Chest* ; Heart* ; Humans ; Male ; Marfan Syndrome* ; Mitral Valve ; Mitral Valve Insufficiency ; Ribs ; Sternotomy ; Thoracic Surgery*

1. It was attempted to clarify the mechanism of the pressor response to raised intracranial pressure in urethane-anesthetized rabbits. 2. Intraventricular clonidine markedly inhibited the pressor response to raised intracranial pressure. 3. Intraventricular regitine antagonized the above mentioned inhibitory effect of clonidine on the pressor response. 4. In reserpine-treated rabbits the pressor response to raised intracranial pressure was not observed, whereas after the intraventricular administration of norepinephrine the pressor response was observed. 5. Intraventricular clonidine inhibited the pressor response that could be observed in the reserpine-treated rabbits after the intraventricular norepinephrine. 6. It is inferred that raised intracranial pressure stimulated some part of the brain to cause the increase of norepinephrine release, resulting in the increase of the sympathetic outflow and the elevation of blood pressure.
Blood Pressure ; Brain ; Clonidine* ; Intracranial Pressure* ; Norepinephrine ; Phentolamine* ; Rabbits*

No abstract available.
Polycystic Kidney Diseases*

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