Pleomorphic xanthoastrocytoma is a rare tumor, presenting superficially over the cerebral hemispheres of young subjects. We report a case of Pleomorphic xanthoastrocytoma of the cervical cord in view of its unusual location. The patient is a 20-year-old female, presenting with left neck pain radiating to the left shoulder one month before the admission. Magnetic resonance imaging revealed a high signal lesion in the upper cervical cord lower brainstem in T2 weighted image. On operation, intramedullary tumor was present and it was relatively well delineated. Near-total removal of the spinal cord mass was done. Microscopically, the tumor was composed of pleomorphic astrocytes, which were spindle-shaped with cytoplasmic processes and hyperchromatic nuclei. Other cells were round with vesicular nuclei and abundant eosinophilic cytoplasm. A few giant cells were seen. Despite the marked pleomorphism, mitosis or necrosis was not encountered. Scattered foamy cells were also seen, which showed negative staining in GFAP immunostaining in contrast to other GFAP-positive tumors cells.
Female ; Humans

Adolescent ; Diseases in Twins ; Female ; Humans ; Hyperthyroidism ; Pregnancy ; Twins, Monozygotic

Cysts of the iris may be primary lesions of either epithelial or stromal in origin, or secondary lesions after trauma or surgery. The stromal cyst is usually of primary lesion, although it could be secondary as shown in our case. We report a case of recurrent iris cyst after trauma. The patient was an 8-year-old girl. She had a penetrating injury of the involved eye three years ago. She had a similar iris cyst removed two years before this surgery. Histologically, the cyst was lined by non-keratinizing stratified squamous epithelia and subjacent fibrovascular tissue. Islands of melanin-containing epithelial cells and acellular basophilic degenerating materials were also seen in the lesion. This case represents a stromal epithelial cyst that by history appears to be of post-traumatic etiology.
Cysts

An intraparotid benign lymphoepithelial cyst is a rare disease characterized by unilateral painless swelling of parotid region. The histogenesis is controversial. Surgical excision is recommended for diagnosis and curative treatment. We present a case of benign lymphoepithelial cyst arising in a patient with neurofibromatosis. A 46-year-old woman presented with a slowly growing multilocular cystic mass in the left cheek. The cystic mass measured 4 cm in maximal outer diameter and the cystic wall was thick and yellowish pale to gray, soft with well circumscribed margin. Microscopically, the multilocular cyst was lined by stratified squamous epithelium for the most part and underlying lymphoid tissue aggregates with follicles and sharply demarcated from adjacent salivary parenchyma which is of normal appearance and without lymphoid aggregates. Since this lesion is absolutely benign, it is important to separate this benign cyst from cystic salivary gland tumors.
Female ; Humans ; Cysts

Nager syndrome is a rare malformation complex characterized by facial, limb, and skeletal morphogenesis.The mode of inheritance has not been definitely established. Major karyotypic abnormalities were seldom associated with this syndrome. We report on an infant with Nager acrofacial dysostosis that was associated with 45,X monosomy. This baby was born to a 36-year-old multigravid woman after 37 weeks of gestation and with maternal hydramnios. The baby girl died of airway obstruction due to retruded tongue 3 hours after birth. Phenotypically, this this patient had mandibulofacial dysostosis, radioulnar synostosis, hypoplasia and aplasia of thumbs, peripheral edema and apparently normal genital organs. We confirmed that major chromosomal anomaly including 45,X monosomy could be associated with Nager syndrome, although its pathogenetic significance remains unanswered.
Adult ; Airway Obstruction ; Dysostoses ; Edema ; Extremities ; Female ; Genitalia ; Humans ; Infant ; Mandibulofacial Dysostosis ; Monosomy* ; Parturition ; Polyhydramnios ; Pregnancy ; Synostosis ; Thumb ; Tongue ; Wills

We report an autopsy case of the brain stem glioma that extended extensively in the brain stem itself and cephalad. This 18-year-old boy first presented with dizziness, vomiting and left side weakness with left facial palsy. Brain MRI revealed a diffusely infiltrative tumor involving whole medulla, pons and lower midbrain. A total of 4000 R was given with some alleviation of respiratory difficulty. He died one year after the onset. Autopsy revealed the tumor involving pons, a portion of medulla oblongata, and cerebellum. The tumor showed diffusely infiltrative pattern and extended along the periventricular area to the thalamus and corpus callosum. The cut surface was grayish white and solid. It also showed areas of myxoid degeneration and necrosis probably related to radiation therapy. Microscopically the tumor was a cellular and pleomorphic glioma that showed some astrocytic differentiation. It was diffuse without geographic necrosis.

A case of Juvenile Granulosa Cell Tumor (JGCT) of the ovary with unusual pleomorphic histologic and malignant biologic behaviour is described. The tumor occurred in a 10-year-old girl and was associated with clinical features of isosexual pseudoprecosity and a marked elevation of serum estradiol. The mass manifested initially in the right ovary and subsequently involved the contralateral ovary. A multi-organ metastasis developed during a 6-month-interval despite chemotherapy. She received two operations at 6-month interval, and tissues were obtained from the tumor mass. A marked histologic difference was observed between these two samples. The second biopsy showed profound cellular pleomorphism with numerous multinucleated tumor giant cell formation and hyaline bodies. The differential diagnosis from germ cell tumor and the possible factors for the pleomorphism are discussed.
Diagnosis, Differential ; Biopsy ; Neoplasm Metastasis

To elucidate a possible prognostic factor, we studied 91 cases of breast carcinoma for the expression of n-tn23 protein using an immunohistochemical method, and compared these results with the known prognostic parameters of the breast carcinoma. The mn23 protein was intensely stained in the cytoplasm and/or the nucleus of carcinoma cells in 82 cases(90.1%). There were two patterns of cytoplasmic staining; heterogeneous pattern and homogeneous pattern. Among the positive cases, 43 cases(47.2%) were heterogeneous while 39 cases(42.8%) were homogeneous. Axillary lymph node metastases(p<0.005) was found more frequently in the heterogeneous pattern group(79.0%) than in the homogeneous pattern group(41.0%). There was no significant correlation between nm23 protein expression and other parameters such as patient age, tumor size, estrogen receptor, histopathologic grade, and p53 overexpression. Although axillary lymph node metastasis was correlated with the disease free status(p<0.0005) and patient survival (p<0.05), they showed no correlation with nin23 expression. Multivariate analysis showed that axillary lymph node metastasis was the only prognostic indicator(p<0.05), and the expression of nm23 protein was of borderline significance. The results suggest that the homogeneous and/or granular cytoplasmic expression of mn23 protein plays a role in the suppression of nodal metastasis in breast carcinoma and might contribute in predicting patient survival.
Neoplasm Metastasis

To elucidate the developmental sequence of the eccrine sweat gland, a morphologic and a morphometric observation were done using developing human embryos and fetuses. Five embryos and sixty four fetuses from the 9th to the 38th week of the gestational age were studied. The skin was sampled in eight different areas, i.e., scalp, forehead, face, chest, abdomen, back, palm and sole. Routine histological sections were made for histological evaluation and morphometric analysis. The results obtained were as follows : The primordia of the eccrine sweat glands appeared first as regular undulation of the basal cells in the palm and the sole in the 13th week of getation. Subsequently, elongation and coiling of the cell cords were noted from the 16th to the 18th weeks. Intraductal lumen formation was first noted in the 20th week. Secretory segment of the eccrine sweat glands were noted from the distal part of the coiling intradermal sweat duct in the 22nd week of the gestational age. The eccrine sweat glands became fully developed by the 28th week of gestation and this included the clear cell, the dark cell and the myoepithelial cell. In the morphometric analysis, the number of eccrine epithelial buddings were decreased with aging and the highest were in the palm and the sole. The diameter of the eccrine sweat duct showed no significant change by gestational age or in the different sites observed. Straight and coiled eccrine sweat ducts or glands were lengthened into the deep reticular dermis and upper portion of the subcutaneous adipose tissue with an increase of the gestational age. The above results suggest that developmental stage and the number of eccrine glands of the skin in the fetal stage is different from other areas of the body, especially in the palm and the sole.
Abdomen ; Aging ; Dermis ; Eccrine Glands ; Embryonic Structures ; Fetus ; Forehead ; Gestational Age ; Humans ; Pregnancy ; Scalp ; Skin ; Subcutaneous Fat ; Sweat Glands* ; Sweat* ; Thorax

To elucidate the developmental sequence of the eccrine sweat gland, a morphologic and a morphometric observation were done using developing human embryos and fetuses. Five embryos and sixty four fetuses from the 9th to the 38th week of the gestational age were studied. The skin was sampled in eight different areas, i.e., scalp, forehead, face, chest, abdomen, back, palm and sole. Routine histological sections were made for histological evaluation and morphometric analysis. The results obtained were as follows : The primordia of the eccrine sweat glands appeared first as regular undulation of the basal cells in the palm and the sole in the 13th week of getation. Subsequently, elongation and coiling of the cell cords were noted from the 16th to the 18th weeks. Intraductal lumen formation was first noted in the 20th week. Secretory segment of the eccrine sweat glands were noted from the distal part of the coiling intradermal sweat duct in the 22nd week of the gestational age. The eccrine sweat glands became fully developed by the 28th week of gestation and this included the clear cell, the dark cell and the myoepithelial cell. In the morphometric analysis, the number of eccrine epithelial buddings were decreased with aging and the highest were in the palm and the sole. The diameter of the eccrine sweat duct showed no significant change by gestational age or in the different sites observed. Straight and coiled eccrine sweat ducts or glands were lengthened into the deep reticular dermis and upper portion of the subcutaneous adipose tissue with an increase of the gestational age. The above results suggest that developmental stage and the number of eccrine glands of the skin in the fetal stage is different from other areas of the body, especially in the palm and the sole.
Abdomen ; Aging ; Dermis ; Eccrine Glands ; Embryonic Structures ; Fetus ; Forehead ; Gestational Age ; Humans ; Pregnancy ; Scalp ; Skin ; Subcutaneous Fat ; Sweat Glands* ; Sweat* ; Thorax