Telomerase is an RNA-dependent DNA polymerase that synthesizes TTAGGG telomeric DNA onto chromosomal ends to compensate for sequence loss during replication. It has been detected in a variety of human malignancies, suggesting that such activity may play a role in the tumorigenic process. To determine whether telomerase is reactivated in malignant fibrous histiocytoma, 12 tissue samples with this tumor were analyzed for the telomerase activity by a radioactive PCR-based TRAP (telomeric repeat amplification protocol) assay. All of the tumors were further investigated for the expression of human telomerase RNA (hTR) by an in situ hybridization (ISH). Telomerase activity was detected in one (8.3%) sample. Expression of hTR was demonstrated in 7 (58.3%): one telomerase-positive and six telomerase-negatives. These data indicate that the reactivation of telomerase is an uncommon event and not an important factor involved in tumorigenesis in malignant fibrous histiocytoma. It is noteworthy that 50% of the patients with grade 2 tumors expressed hTR, suggesting that telomerase RNA may be useful as a marker for identifying tumor aggressiveness earlier than the conventional histopathologic grading scale.
Carcinogenesis ; DNA ; Histiocytoma, Malignant Fibrous* ; Humans ; In Situ Hybridization ; RNA* ; RNA-Directed DNA Polymerase ; Telomerase*

An eccrine squamous syringometaplasia (ESS) is defined as a mature squamous metaplasia of the eccrine ducts. The clinical and pathological features of an ESS are presented. Syringometaplasia is a rare lesion, mostly occuring in the extremities, and as far as we know, no report on the ESS has been published in Korean literature. We experienced a case of an ESS occured in a 15 year-old male, who had a tender erythematous plaque in the right knee. The histologic examination revealed some scattered nests of metaplastic squamous epithelium in the deep dermis, associated with acute nonspecific panniculitis. The importance of the ESS is that it histologically simulates the well differentiated squamous cell carcinoma. The histopathologic findings were discussed and a brief review of the literature was made.
Adolescent ; Carcinoma, Squamous Cell ; Dermis ; Epithelium ; Extremities ; Humans ; Knee ; Male ; Metaplasia ; Panniculitis

This case report describes a paraganglioma of the cauda equina in a 37-year-old man, as documented by light microscopy and immunohistochemistry. The patient experienced low back pain of 3 years duration, with the recent onset of sciatic pain and altered sensation in the right leg. Magnetic resonance imaging of L4 vertebral level revealed an ovoid, solid mass in the cauda equina. The mass was measured 1.5 cm in the greatest diameter. The histologic appearance was characterized by organoid pattern with clusters of chief cells (zellballen). Immunohistochemically, tumor cells are positive for keratin, epithelial membrane antigen, vimentin, neuron specific enolase and chromogranin.
Adult ; Cauda Equina* ; Humans ; Immunohistochemistry ; Leg ; Low Back Pain ; Magnetic Resonance Imaging ; Microscopy ; Mucin-1 ; Organoids ; Paraganglioma* ; Phosphopyruvate Hydratase ; Sensation ; Vimentin

Arteriovenous malformation of the uterus is rarely encountered. It is potentially lethal because of profuse, uncontrollable bleeding at the time of diagnostic dilatation and curettage. To our knowledge, only 38 cases of localized uterine arteriovenous malformation have been reported in English and Korean literature. Recently we experienced a case of uterine arteriovenous malformation in a 28-year-old multiparous woman. Under the clinical impression of uterine tumors or arteriovenous malformation, a total hysterectomy was done. Gross examination demonstrated a vascular nature of the mass in the posterior wall of the fundus. Microscopically, there was an ill-defined mass composed of numerous irregular, anastomosing large vessels with various diameters.
Female ; Humans

The eccrine poroma was described first by Pinkus et al. in 1956 as a subgroup of benign solid hidradenoma with the histologic structure resembling acrosyringium. The histologic appearance of eccrine poroma greatly resembles that of seborrheic keratosis and basal cell carcinoma. But the clinical lesion is fairly unique and the tumor is most commonly found almost exclusively on the foot, in the skin of the plantar surface. During the past four years, the authors experienced five cases of eccrine poroma which were diagnosed by histopathological examination of the tumor mass. All the cases occurred in the sole and lateral sides of the foot as well. Clinical and pathological features were reviewed and a brief review of the literatures was done.

Linear nevus sebaceous syndrome is an uncommon neurocutaneous disorder characterized by nevus sebaceus, seizures, and mental retardation, and is often accompanied by ocular anormalies. We recently experienced a case of conjunctival choristoma associated with linear nevus sebaceous syndrome in a 17-month-old boy. The child was the product of a full-term uncomplicated pregnancy and normal delivery with normal karyotype of 46, XY. He was noted at birth to have a conjunctival mass on the right eye and linear nevi over the midline facial region and right temporoparietal scalp. Ophthalmologic examination revealed optic atrophy, peripapillary staphyloma, macular hypogenesis, and osseous choristoma of the choroid. A computed tomography scan demonstrated widening of the subarachnoid space and moderate atrophy of the cerebral and cerebellar hemispheres. He has been followed by ophthalmology and completely free of seizures since birth. The patient underwent excision of the conjunctival mass and biopsies of the skin lesion at 17 months of age. Histologic examination of the skin biopsies was characteristic for the linear sebaceous nevus of Jadassohn. Histologic findings of the conjunctival lesion showed a complex choristoma that contained lacrimal gland tissue and cartilage.
Atrophy ; Biopsy ; Cartilage ; Child ; Choristoma* ; Choroid ; Humans ; Infant ; Intellectual Disability ; Karyotype ; Lacrimal Apparatus ; Male ; Neurocutaneous Syndromes ; Nevus* ; Nevus, Sebaceous of Jadassohn ; Ophthalmology ; Optic Atrophy ; Parturition ; Pregnancy ; Scalp ; Seizures ; Skin ; Subarachnoid Space

Orbital mesenchymal chondrosarcoma, first described by Luis et. al in 1971, is a very rare tumor of characteristic histologic features. A 21-year-woman was admitted with a 4-month histoiry of rapidly progressive proptosis and visual disturbance. Right orbital exenteration was performed under the clinical diagnosis of orbital calcifying tumor. Grossly, the tumor presented as a multibloblated, circumscribed mass that measures 5.5 cm in the greatest dimentsion. Cut sections resembled ordinary chonrosarcoma. Microscopically, the tumor was composed of undifferentiated mesenchymal cells, interspersed nodules of well differentated cartilagenous tissue, areas of gradual transition from undifferentiated mesenchymal cells to cartilage, and hemangiopericytoma-like areas. A brief summary of the histopathological aspect of this tumor and a review of literature are presented.
Female ; Humans

No abstract available.
Antibodies, Antineutrophil Cytoplasmic* ; Incidence*

Tumorlet is a rare lesion of disputed origin that was first described by whitwell in 1955, and about one-third of the reported cases have been associated with underlying lung disease. Patient was a 60-year-old female who was admitted with a histroy of chest discomfort and dyspnea. Right lower lobe was partially resected under the clinical diagnosis of the bronchogenic cyst. Grossly, lung tissue around round cystic lesion appeared brown firm and somewhat fibrotic, and showed several scattered ill-defined whitish gray nodules. Microscopically, lung tissue around bronchogenic cyst was partially obliterated by dense fibrous scar tissue. Within this areas of fibrosis, and in the wall of alveolar ducts and respiratory bronchioles, innumerable microscopic tumorlets were found and argyrophilic granules were also demonstrated in scattered tumorlets with Grimelius stain.
Female ; Humans ; Cysts

Agenesis of the dorsal pancreas is one of the rare congenital anomalies of the pancreas. Six cases of them have been reported. We have experienced an autopsy case of agenesis of the dorsal pancreas associated with fetal death in the uterus. Grossly, the body and tail of the pancreas and uncinate process were not found and those were partially replaced by adipose tissue. No abnormality was noted in the other organs. Microscopically, pancreatic tissue with autolytic change was identified only in the head portion of the pancreas.