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

Lymphoid tumors of the orbit are rare, and sometimes it is not possible either clinically or histologically to differentiate between lymphoid tumor and pseudolymphoma. Some authors assert that the degree of cytologic differentiation appears to be the single most important factor for determining the prognosis of patients with orbital lymphoid lesions. However, the cytomorphologic basis is not so helpful to diagnose and classify our case, which shows some discrepancy between pathological findings and clinical and radiological findings. At first we misdiagnosed our case as orbital malignant lymphoma on the basis of cytomorphology and immunohistochemical study. But no responce to local intensive radiotherapy and the follow up study of the patient suggest orbital pseudolymphoma.
Follow-Up Studies

Trichoblastic fibroma is a benign trichogenic tumor that has both epithelial and mesenchymal components and exhibits partial follicular induction. We studied 4 cases of trichoblastic fibroma and reviewed their clinical and histologic features. Two tumors were present in the face. The remaining two were in the vulva and perianal area, respectively. The age of the patients ranged from 53 to 68 years, with an average age of 62. All were female. Histologically, the lesions showed a well circumscribed mass, located at dermo-subcutaneous junction in three patients and subcutaneous in one. They demonstrated mesenchymal induction evidenced by hair germ-like structure and perifollicular sheath. There was no connection between the tumor and epidermis. Differentiation toward hair structure led to the formation of the infundibulum through inner root sheath. Trichoblastic fibroma may be confused clinically and/or histologically with basal cell carcinoma. Identification of the mixed epithelial and mesenchymal components, and the absence of epidermal connection and cleft within the stroma are important in differentiating this benign neoplasm from basal cell carcinoma.
Carcinoma, Basal Cell ; Epidermis ; Female ; Fibroma* ; Hair ; Humans ; Vulva

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

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.

Primary malignant parovarian epithelial tumors are extremely rare, with only 56 cases previously reported in the world literature. Most parovarian epithelial tumors are of paramesonephric (Mullerian) origin. In this article, we report the first case in the Korean literature of papillary serous cystadenoma of borderline malignancy from paramesonephric parovarian cyst. This case presented here is of particular interest since this neoplasm is rare. A 48-year-old female underwent a hysterectomy with bilateral adnexectomy and pelvic lymph node dissection for a cystic tumor of the right parovarian area. The tumor, measuring 13 cm in diameter, was apart from the ovaries, fallopian tubes and uterus. The cyst wall had well-developed smooth muscle layers. The cyst was lined with mildly atypical ciliated and non-ciliated low columnar cells compatible with those of paramesonephric origin. From the inner surface of the cyst several cauliflower-like structures projected into the lumen. They were of a predominantly papillary architecture covered by atypical epithelial cells with piling-up and occasional glandular growth. No destructive stromal invasion was identified.
Cystadenoma, Serous* ; Epithelial Cells ; Fallopian Tubes ; Female ; Humans ; Hysterectomy ; Lymph Node Excision ; Middle Aged ; Muscle, Smooth ; Ovary ; Parovarian Cyst* ; Uterus

Compared with osteosarcoma of bone, primary osteosarcoma of the soft tissue is very rare. Extraskeletal osteosarcoma is also a highly malignant pleomorphic sarcoma composed of cells exhibiting primarily osteoblastic, and to a lesser extent, chondroblastic differentiation. A case of extraskeletal osteosarcoma in the right sole is presented in a 67 year old male. The patient had noticed a progressively enlarging soft tissue mass, during about 14 months. This sarcoma was located in the soft tissues without attachment to the skeleton, as determined by examination of the X-Ray findings. The mass of right plantar portion was simply excised and pathologically confirmed to be an extraskeletal osteosarcoma. The clinical and pathological features of this sarcoma are described, and brief review of the literature is made.

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.

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