Folliculosebaceous Cystic Hamartoma in a Patient with Neurofibromatosis Type I

Folliculosebaceous cystic hamartoma (FSCH) is a rare cutaneous hamartoma composed of dilated folliculosebaceous units and mesenchymal elements. It presents as a papule or nodule usually on the face and scalp, rarely on the genital or trunk area. Histologically, FSCH shares several similar features to sebaceous trichofolliculoma. We report one case of FSCH misdiagnosed as a neurofibroma. He was a 38-year-old man with a neurofibromatosis type I and a nodule on his left earlobe was excised under the impression of neurofibroma. Pathological examination revealed FSCH. Although FSCH is clinically not distinctive, awareness of the lesion is important to differentiate papulonodular or cyst-like cutaneous lesions.

Laparoscopic Enucleation of a Nonfunctioning Neuroendocrine Tumor of the Pancreas

Laparoscopic approaches are increasingly used in pancreatic surgery. In the treatment of neuroendocrine tumors (NETs) of the pancreas, enucleation is one of the recommended surgery. Although many clinical experiences have reported the safety and efficacy of laparoscopic enucleation of functioning NETs, such as insulinomas, few reports have explored such treatment for non-functioning NETs. Here, we present a case of 70-year old female patient who underwent successful laparoscopic enucleation of a nonfunctioning NET located in the body of the pancreas.

Utility of In-House PCR for HLA-B27 Typing: Comparison of Concordance Rate between PCR Kit and In-House PCR / 대한진단검사의학회지

BACKGROUND: Commercial kits of PCR method are widely used in HLA-B27 typing; however, their cost is relatively high. In this study, we evaluated the utility of an in-house PCR method by comparing it with that of a commercial kit. METHODS: HLA-B27 typing was done in 188 patients by using two PCR methods, Absolute(TM) HLAB27 PCR kit (Biosewoom, Korea) and an in-house PCR method. The primers used in the in-house method were prepared by Bioneer (Korea). Both PCR tests were done by Gene Amp PCR System 9600 (Perkin-Elmer Centus Corp., USA). RESULTS: The commercial kit and in-house PCR showed 100% concordance rate with each other in HLA-B27 typing. Of 188 patients tested 72 (38.3%) were positive and 116 (61.7%) were negative by the both tests. Of 62 patients with ankylosing spondylitis, 50 were positive (80.7%). CONCLUSIONS: The in-house PCR is a reliable and cost-effective method and can replace or supplement commercial kits for HLA-B27 typing.

A Case of Solitary Fibrofolliculoma of the Auricle / 대한이비인후과학회지

Fibrofolliculoma is a relatively uncommon benign neoplasm that develops on the head and neck, upper trunk and arm. Typically, it presents as a multiple lesion, and each has a characteristic histopathologic pattern of centrally dilated follicular infundibulum surrounded by well-circumscribed, thick mantle of fibrotic tissue and numerous thin anastomosing strands of follicular epithelium extending into stroma. We recently confirmed a unique case of solitary nodular form of fibrofolliculoma occurred on the cavum concha of 56-year-old male patient. We report this interesting case with a review of literature.

A Case of Histiocytoid Sweet's Syndrome with Myelodysplastic Syndrome / 대한피부과학회지

Sweet's syndrome is an acute febrile neutrophilic dermatosis that occurs with malignant disease, mainly myeloid hemopathies, in about 20% of cases. When associated with myelodysplasia, clinical and histologic features of Sweet's syndrome can be atypical; however its significance is still debated. We encountered a case of Sweet's syndrome associated with myelodysplastic syndrome in a 63-year-old woman. The clinical presentation strongly suggested Sweet's syndrome but, histopathologic examination showed dense histiocytic infiltration with prominent upper dermal edema and little neutrophil infiltration. In the evaluation for atypical histopathology and laboratory abnormalities, she was diagnosed with myleodysplastic syndrome by peripheral blood smear and bone marrow biopsy. Histiocytoid Sweet's syndrome is a currently accepted concept and probably has a close relation with underlying myeloid hemopathies.

The Effects of Testosterone on Skin Barrier / 대한피부과학회지

BACKGROUND: Although there are no known gender-related differences in permeability barrier function in adults, estrogen accelerates whereas testosterone retards barrier development in fetal skin. However, there have been few studies concerning the effects of testosterone on the skin barrier. OBJECT: We evaluated the effects and mechanisms of testosterone on the skin barrier. METHODS: In this experiment, hairless mice were divided into three groups; sham-operated, castrated and testosterone-replacement castrated group. Testosterone was administered subcutaneously once a day for 7 days. We performed a skin biopsy at 7 days and performed hematoxyline-eosin staining, calcium-ion capture cytometry and the immunohistochemical examination of involucrin, loricrin, filaggrin and proliferating cell nuclear antigen (PCNA). The specimens were prepared for electron microscopy using RuO4 and OsO4 postfixation. RESULTS: The results were summarized as follows 1. Light microscopic findings of the testosterone-replacement castrated group showed apparent hyperkeratosis and acanthosis, not present in the sham-operated and castrated group. 2. Whereas the expression of involucrin, loricrin and filaggrin of immunohistochemical staining and in situ hybridization of the sham-operated and castrated group were normal, it was abnormal in the testosterone-replacement castrated group. 3. Labelling indices for PCNA in the sham-operated and castrated group were not statistically different, but the testosterone-replacement castrated group showed a marked increase of PCNA labeling index. 4. Wherease the calcium gradient was normal in the sham-operated and castrated group, it was distorted in the testosterone-replacement castrated group. Calcium deposition was increased through all layers of the epidermis and the calcium gradient disappeared in the testosterone-replacement castrated group. 5. Normal looking membrane structure was observed in the sham-operated and castrated group, but a membrane structure which appeared fragmented, incomplete lipid bilayer structures and prominent dilatation of lacunar domains were observed only in the testosterone-replacement castrated group. CONCLUSION: From the above results, it is concluded that there is a functional alteration of the epidermal barrier induced by testosterone, including the formation of an abnormal cornified envelope and also incomplete lipid synthesis.

A Case of Placenta Percreta during First Trimester / 대한산부인과학회지

Placenta percreta is a life-threatening complication of pregnancy, and it is very rarely noted to occur in the first trimester. We present here a case of placenta percreta with a missed abortion that occurred at 12 gestational weeks. During curettage, hysterectomy was required due to the heavy bleeding. On operative finding, hemorrhagic placental tissue in the lower uterine segment. It is extending into the myometrium and periuterine soft tissue. Histological examination revealed features of placenta percreta.

Detection of Core Gene Deletion and Its Clinical Significance in Chronic Hepatitis B Virus infection / 대한진단검사의학회지

BACKGROUND: In the course of chronic hepatitis B virus (HBV) infections, a point mutation or core gene deletion has been attributed to play a role in persistence of HBV infection. This study was undertaken to determine the prevalence of core gene deletion in chronic HBV infections, molecular characteristics, and its clinical significance. METHODS: Among 276 patients with positive results in HBV PCR for precore and core region, patients with smaller bands in addition to the band of expected wild type size, suggestive of deletion, were analyzed by direct sequencing, and hospital records were reviewed on 217 patients. RESULTS: The prevalence of core gene deletion among patients with positivity in HBV PCR was 12.7% (35/276) and they always existed together with wild type. Seventeen patients were further studied by direct sequencing and 16 patients had similar positions of deletion in the center of core gene (nt 2000-2200). Of 35 patients with core gene deletion, 26 (74.3%) were HBeAg-positive and 7 (20.0%) were anti-HBe-positive. There were no significant differences in AST, ALT and HBV DNA quantitation between the wild type and the deletion groups. CONCLUSIONS: HBV core gene deletion is frequently found in chronic HBV infections and has some common features in deleted position, serologic markers and clinical state. However, the mechanism and time at which deletion mutants appear and disappear, and their clinical significances are not fully understood. By longitudinal studies for chronic hepatitis B patients, we should be able to demonstrate the immunologic significance of the core gene deletion mutation.

Cytologic Features of Secretory Meningioma in Squash Preparation: A Case Report / 대한세포병리학회지

Secretory meningioma is a distinct subtype of meningioma. We describe the cytologic features of a secretory meningioma on squash preparations, in comparision with other cytologic mimickers. A 54-year-old woman presented with hearing loss, vertigo, tinnitus, and headache for seven years. A brain MRI study revealed a 4.5 cm sized mass in the cerebellopontine angle, which showed homogenous signal intensity in T2-weighted image. The intraoperative squash smear showed some well-defined, thin rimmed intracytoplasmic inclusions, containing a finely granular eosinophilic core among less cohesive meningiomatous cells. Histologic sections revealed a meningothelial meningioma with scattered inclusions, with periodic acid-Schiff, carcinoembryonic antigen, and cytokeratin positivity. Identification of characteristic intracytoplasmic inclusions is helpful for diagnosing secretory meningiomas. On squash preparations, differential diagnoses included tumors with inclusions or cytoplasmic vacuolizations, such as metastatic mammary infiltrating ductal carcinoma, gastric adenocarcinoma, hepatocellular carcinoma, and clear cell ependymoma, oligodendroglioma, hemangioblastoma, chordoma, and other variants of meningiomas (clear cell, xanthomatous, microcytic, and chordoid variants). In addition, the possibilities of glioma with eosinophilic granular body, and metastatic tumors from mammary infiltrating ductal carcinoma, gastric adenocarcinoma, and hepatocellular carcinoma in meningioma should be considered.

Macrofollicular Variant of Papillary Thyroid Carcinoma with Extensive Hemorrhage / 대한세포병리학회지

BACKGROUND: The macrofollicular variant of papillary thyroid carcinoma (MVPC) is characterized by macrofollicles occupying more than half of the tumor and demonstrating nuclear features of classic papillary carcinoma. It is difficult to recognize on fine needle aspiration (FNA) cytology due to the paucity of aspirated neoplastic cell clusters, especially when the tumor is associated with extensive areas of hemorrhage. CASE: A 34-year-old female presented with a well-demarcated nodule in the thyroid gland, diagnosed as a benign nodule on ultrasonography and computed tomography. FNA cytology smear revealed a few small aggregates of follicular cells with morphological features suspicious for papillary carcinoma, set in a background of hemorrhage, inflammatory cells, and hemosiderin-laden macrophages. Intraoperative frozen section revealed macrofollicular nests filled with hemorrhage and composed of follicular cells demonstrating nuclear clearing and grooves. CONCLUSION: MVPC is a rare but distinctive variant of papillary carcinoma, which is easily mistaken for adenomatous goiter or benign macrofollicular neoplasm on radiologic findings. The cytopathologist should alert oneself on encountering benign radiologic findings and any smear composed of scant numbers of follicular cells with nuclear features suspicious for papillary carcinoma despite the bland-looking background of hemorrhage and hemosiderin-laden macrophages, and recommend intraoperative frozen sections for a definite diagnosis.

A Case of Thymolipoma Simulating Cardiomegaly / 결핵

Thymolipoma is rare benign tumor of the thymic gland and mostly occurs at anterior mediastinum. Thymolipoma comprises 2~9% of thymic tumor and less than 1% of mediastinal mass. Therefore, thymolipoma should be differentiated from anterior mediastinal tumor such as thymoma, germ cell tumor and lymphoma. These tumors resemble cardiomegaly, pleural effusion, basal atelectasis, pericardial tumor and cyst, pleural tumor, lung cancer and pulmonary sequestration, and differentiated from above mentioned diseases. Though most cases are asymptomatic, there can be dyspnea with compression of adjacent organ by mass effect, and myasthenia gravis. We experienced a thymolipoma simulating cardiomegaly and report the case with the review of literatures.

Expression of Anaphase Promoting Complex (APC) and APC Regulatory Proteins in Invasive Ductal Carcinoma Associated with Paget's Disease

BACKGROUND: Oncogene expression in Paget's disease of the breast is not well known. To characterize invasive ductal carcinoma associated with Paget's disease, we studied expression of anaphase promoting complex (APC) with its regulatory proteins. METHODS: Immunohistochemical stainings were done with 10 cases of invasive ductal carcinoma associated with Paget's disease for APC, pituitary tumor transforming gene (PTTG), cyclin B1, p53, cyclin D1, and c-erbB-2. The expressions of these markers in Paget's disease were compared with those in the associated with carcinoma. RESULTS: APC, PTTG, cyclin B1, and c-erbB-2 were positive in all of the cases with both Paget's disease and underlying carcinoma. p53 was expressed in Paget's disease of 6 cases (60%) and in carcinoma of 7 cases (70%). Cyclin D1 was positive in Paget's disease of 8 cases (80%) and in carcinoma of 9 cases (90%). CONCLUSIONS: Breast carcinomas with Paget's disease seem to be distinguished by the high expression of APC, cyclin B1, PTTG, c-erbB2, and cyclin D1 in contrast to breast cancers without Paget's disease. Furthermore, the similar expression patterns of APC and APC regulatory proteins in both Paget's disease and underlying breast cancer support the epidermotropic theory as its pathogenetic mechanism.

Drug-induced Extraocular Myotoxicity Associated with Diplopia after Cataract Surgery

PURPOSE: To assess the myotoxicity of local anesthetics and antibiotics which are considered as the main causes of diplopia and extraocular motility disturbance following cataract surgery. METHODS: A total of 48 rabbits were classified into 4 groups. The control group received an injection of 0.3 ml normal saline in the subconjunctival space above the superior rectus muscle. The first experimental group received an injection of the same amount of a local anesthetic (2% lidocaine) in the same manner as the control group. The second group received an injection of 0.3 ml of a local anesthetic directly into the extraocular muscle (superior rectus muscle), the third group received an injection of 0.3 ml of an antibiotic (gentamicin) in the subconjunctival space above the superior rectus muscle. Histologic and electron microscopic changes following injections were compared over time in each group. RESULTS: Light microscopic findings showed that subconjunctival injections of antibiotics caused the most severe inflammatory and fibrotic reactions of the muscular fascicle, whereas subconjunctival injections of local anesthetics led to the least damage of the muscular fascicle and less infiltration of inflammatory cells. Changes in histological findings showed that necrotic muscle fibers and inflammatory cell infiltration at 1 week following injections. Basophilic myogenous satellite cells indicating regeneration of the damaged muscles appeared thereafter. At 2 and 4 weeks, the group injected with antibiotic showed local and consistent infiltration of inflammatory cells and fibrosis, whereas the groups injected with anesthetics showed regeneration of most of the damaged fascicle. CONCLUSIONS: In terms of temporary or permanent diplopia following an injection of local anesthetics or antibiotics after cataract surgery, toxicity of anesthetics or antibiotics on extraocular muscle is the main cause from these Results.

A case of parathyroid carcinoma with meningioma / 대한내과학회지

Parathyroid carcinoma is a very rare disease which comprising 0.1~5% of hyperparathyroidism, and it usually presents with marked hypercalcemia. Clinically, it shows hypercalcemia due to the effect of excessive secretion of parathyroid hormone, bone disease, renal involvement and palpable neck mass. It is known that hyperparathyroidism is induced mostly by parathyroid adenoma but it can be seen in parathyroid hyperplasia, hyperparathyroid carcinoma, rarely associated with familial or multiple endocrine neoplasia. Parathyroid carcinoma can be diagnosed with distant metastasis or local invasion. Treatment is complete resection of primary cancerous lesion and local tissue. Since recurrence or distant metastases are frequent, radiological studies should be done when hypercalcemia is recurred. Sometimes, other tumors can causes hypercalcemia. There are several reports of parathyroid cancer associated with multiple endocrine neoplasia, but has never been reported of parathyroid carcinoma associated with meningioma. We experienced a parathyroid carcinoma with meningioma in 68 year old woman and report the case with the review of literatures.

A case of parathyroid carcinoma with meningioma / 대한내과학회지

Parathyroid carcinoma is a very rare disease which comprising 0.1~5% of hyperparathyroidism, and it usually presents with marked hypercalcemia. Clinically, it shows hypercalcemia due to the effect of excessive secretion of parathyroid hormone, bone disease, renal involvement and palpable neck mass. It is known that hyperparathyroidism is induced mostly by parathyroid adenoma but it can be seen in parathyroid hyperplasia, hyperparathyroid carcinoma, rarely associated with familial or multiple endocrine neoplasia. Parathyroid carcinoma can be diagnosed with distant metastasis or local invasion. Treatment is complete resection of primary cancerous lesion and local tissue. Since recurrence or distant metastases are frequent, radiological studies should be done when hypercalcemia is recurred. Sometimes, other tumors can causes hypercalcemia. There are several reports of parathyroid cancer associated with multiple endocrine neoplasia, but has never been reported of parathyroid carcinoma associated with meningioma. We experienced a parathyroid carcinoma with meningioma in 68 year old woman and report the case with the review of literatures.

Relationship between Expression of Anaphase-promoting Complex and Prognostic Factors in Invasive Ductal Carcinoma of Breast

BACKGROUND: The role of the anaphase-promoting complex (APC) is to promote the degradation of mitotic cyclins and other substrates involved in sister chromatid adhesions. The APC appears to be responsible for the degradation of cyclin B and may have a potential role in the loss of control concerning cell proliferation in mammalian cells. However, a direct link between the defects in the APC components and oncogenesis has not been estabilished. This study investigates the relationship between APC expression and variable prognostic factors in invasive ductal carcinoma of the breast. METHODS: We evaluated 108 cases of invasive ductal carcinoma surgically resected from January, 1996 to May, 2000 at Wonju Christian Hospital, Wonju College of Medicine, Yonsei University. Immunohistochemical stains for APC, estrogen receptor, and Ki-67 were done in paraffin sections using the avidin-biotin complex method. The results were compared with clinical and pathologic parameters and flow cytometric DNA analysis factors. RESULTS: Forty cases (37.0%) showed immunopositive reactions for APC. The APC positivity in histologic grades 1, 2, and 3 were 28 cases (84.4%), 33 cases (60.0%), and 7 cases (35.0%), respectively (p=0.0011). The APC expressions in cases with the number of mitosis of less than 10, 10-19, and more than 20 per 10 high power fields, were noted in 37 cases (75.5%), 26 cases (63.4%), and 5 cases (27.8%), respectively (p=0.0016). The mean value of the Ki-67 labeling index was 221.7 in the APC-positive group and 317.9 in the APC-negative group (p= 0.0091). DNA flow cytometric analysis revealed higher APC expressions in cases with diploid patterns (p=0.0095). The APC expression rate increased significantly with decreasing histologic grade, with decreasing mitotic activity, in cases with a low Ki-67 labeling index, and those in the diploid group (p<0.05). The APC expression was not statistically correlated with clinical stage, tumor size, and estrogen receptor status. CONCLUSIONS: These findings suggest that positive APC expression may be considered as a good prognostic factor of invasive ductal carcinoma, and loss of APC expression may be related with the progression of breast cancer.

A case of nesidiodysplasia in adult with hyperinsulinemic hypoglycemia / 대한내과학회지

Nesidiodysplasia encompasses increased, maldistributed, and/or, improperly regulated or programmed endocrine cells associated with a clinical endocrine dysfunction. It is a frequent cause of hyperinsulinemic hypoglycemia in neonates and infants, but rare in adults. A 63 year-old woman was admitted due to the loss of consciousness and intense sweating. Hyperinsulinemic hypogiycemia was diagnosed. Under the presumptive diagnosis of insulinoma, abdominal ultrasonography and CT scan were done, but no definite tumor was found. Only a suspicious gradient in insulin concentration was found around the body of the pancreas by percutaneous portal venous sampling. While an exploratory laparotomy was performed, an intraoperative sonography was done. As a result, cystic mass like lesion was found. Then mass enucleation was done. Pathologic examination of the enucleated pancreas revealed no mass and the characteristic nesidiodysplasia. However, after the operation, hypoglycemic symptoms were repeated. So we are instructing her to eat meals frequently.

The Fine Needle Aspiration Cytologic Features of Apocrine Carcinoma of the Breast: A Case Report / 대한세포병리학회지

Apocrine carcinoma of the breast is a very rare subtype. Although it has no clinical differences from usual ductal carcinoma of the breast, it should be categorized as a subtype of breast carcinoma because the cells of apocrine carcinoma reveal characteristic abundant eosinophillic cytoplasms with intraductal apical snouting as well as round or oval nuclei and central macronucleoli. On fine needle aspiration cytology, the cells of apocrine carcinoma have a lot of similarity to benign or reactive apocrine cells of the breast. Therefore, it is difficult to make a differential diagnosis of apocrine carcinoma from mammary neoplasms with similar cytologic findings unless the subtle cytologic differences are recognized. We report the cytologic and histologic findings of a case of apocrine carcinoma in the breast of a 40-year-old female patient. After the fine needle aspiration cytology, she received the lumpectomy and lymph node dissection. The cellularity was moderate to high. The cytoplasmic borders of tumor cells of three-dimensional clusters were relatively distinctive, and the cytoplasm was abundant, eosinophilic, and granular. Although the nuclear/cytoplasmic ratio was low, the nuclei of the cells were variable in size and shape with prominent macronucleoli. Histologically, it was a typical invasive apocrine carcinoma, showing numerous cytoplasmic lysosomes and mitochondriae on electron microscopy.

Fine Needle Aspiration Cytology of Kimura's Disease of Parotid Gland: Report of A Case Cytologically Failed to Diagnose as Kimura's Disease / 대한세포병리학회지

Kimura's disease is a chronic inflammatory disorder of unknown cause and is most prevalent among Asians. The cytologic findings of Kimura's disease are significant numbers of eosinophils in a background of lymphoid cells, occasional fragments of collagenous tissue, proliferation of vessels, and Warthin-Finkeldey polykaryocytes. Among these features, the most important cytologic feature of Kimura's disease is a significant numbers of eosinophils. We experienced a case of Kimura's disease in the parotid gland which we failed to recognize on cytology due to the apparent paucity of eosinophils. On careful retrograde reviewing of the cytologic findings, a few scattered leukocytes, previously interpreted as polymorphous leukocytes, had bilobed nuclei and coarse green but granular cytoplasm on Papanicolaou preparation. These leukocytes showed obvious orange-red intracytoplasmic granules as in eosionophils on Giemsa stain. The paucity of eosinophils may be due to the thick fibrosis around lymphoid follicles or any technical error during aspiration. Whereas the Warthin-Finkeldey type giant cell is not a sensitive cytologic marker of Kimura's disease, it may be a helpful cytologic feature. To reach a correct cytologic diagnosis of Kimura's disease, it is important to keep in mind that searching for Warthin-Finkeldey type giant cells and evaluation of Giemsa stain for detection of eosinophils would be helpful.

Basal Cell Carcinoma with Matrical Differentiation

Basal cell carcinoma (BCC) may exhibit diverse differentiation. In BCC with matrical differentiation, islands of shadow cells, which are characteristic of a pilomatricoma, are located within the tumor. This is an extremely rare variant and to our knowledge, only two review articles and one case report have been published. We recently experienced a case of BCC with matrical differentiation in a 45-year-old Caucasian male. This tumor should be differentially diagnosed from other tumors, particularly a pilomatricoma and pilomatrix carcinoma.