Isolated extramedullary relapse (EMR) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in acute myeloid leukemia (AML) is relatively rare and the clinical significance is still not well characterized, particularly in children. During or after chemotherapy, an uncommon event termed lineage switch, in which the lineage at onset of leukemia converts to another lineage at a later time, is observed in a subset of leukemia patients, but it is a rare event. We report a 10-year-old boy with isolated mediastinal EMR after allo-HSCT for AML who subsequently experienced BM relapse with the cell lineage switching from AML to T-cell acute lymphoblastic leukemia.

We report a rare case of hilar squamous cell carcinoma. A 62-year-old Korean woman complaining of nausea was referred to our hospital. Her biliary computed tomography revealed a 28 mm-sized protruding solid mass in the proximal common bile duct. The patient underwent left hemihepatectomy with S1 segmentectomy and segmental excision of the common bile duct. Microscopically, the tumor was a moderately differentiated squamous cell carcinoma of the extrahepatic bile duct, without any component of adenocarcinoma or metaplastic portion in the biliary epithelium. Immunohistochemically, the tumor was positive for cytokeratin (CK) 5/6, CK19, p40, and p63. Squamous cell carcinoma of the extrahepatic bile duct is rare. To date, only 24 cases of biliary squamous cell carcinomas have been reported. Here, we provide a clinicopathologic review of previously reported extrahepatic bile duct squamous cell carcinomas.
Adenocarcinoma ; Bile Ducts, Extrahepatic ; Carcinoma, Squamous Cell ; Common Bile Duct ; Drug Therapy ; Epithelial Cells ; Epithelium ; Female ; Hepatic Duct, Common ; Humans ; Keratins ; Klatskin Tumor ; Mastectomy, Segmental ; Middle Aged ; Nausea

OBJECTIVE: To investigate the change of maternal characteristics, delivery and neonatal outcomes in gestational diabetes mellitus (GDM) over recent 10 years and to identify the risk factors associated with adverse outcome. METHODS: Consecutive GDM patients (n=947) delivered in our institution were included. Research period was arbitrarily divided into 2 periods (period 1: from 2006 to 2010, period 2: from 2011 to 2015). Multiple pregnancies or preexisting diabetes were excluded. Maternal baseline characteristics, delivery and neonatal outcomes were reviewed. Fetal biometric findings by prenatal ultrasonography were collected. Adverse pregnancy outcome (APO) was defined by the presence of one of the followings; shoulder dystocia, neonatal macrosomia (>4 kg), neonatal hypoglycemia (< 35 mg/dL), respiratory distress syndrome (RDS), and admission to the neonatal intensive care unit (NICU) in term pregnancy. RESULTS: Period 2 was associated with older maternal age (34 vs. 33, P < 0.001) and higher proportion of GDM A2 compared to period 1 (30.9% vs. 23.0%, P=0.009). By univariate analysis, APO was associated with increased body mass index (BMI) at pre-pregnancy (23.4 kg/m² vs. 21.8 kg/m², P=0.001) or delivery (27.9 kg/m² vs. 25.8 kg/m², P < 0.001), higher HbA1c at diagnosis (5.6% vs. 5.3%, P < 0.001) or delivery (5.8% vs. 5.5%, P=0.044), and larger fetal biometric findings (abdominal circumference [AC] and estimated fetal weight, P=0.029 and P=0.007, respectively). Multivariate analysis showed pre-pregnancy BMI (odds ratio [OR], 1.101; 90% confidence interval [CI], 1.028–1.180) and fetal AC (OR, 1.218; 90% CI, 1.012–1.466) were independently associated with adverse outcomes. CONCLUSION: Our study demonstrated the trends and relevant factors associated with the adverse outcomes.
Body Mass Index ; Diabetes, Gestational* ; Diagnosis ; Dystocia ; Female ; Fetal Macrosomia ; Fetal Weight ; Humans ; Hypoglycemia ; Infant, Newborn ; Intensive Care, Neonatal ; Maternal Age ; Multivariate Analysis ; Pregnancy ; Pregnancy Outcome ; Pregnancy, Multiple ; Respiratory Distress Syndrome, Newborn ; Risk Factors* ; Shoulder ; Ultrasonography, Prenatal

A 61-year-old woman was referred to surgery for incidentally found colonic polyps during a health examination. Physical examination revealed widespread eczematous skin lesion without pruritus in the perianal and vulvar area. Abdominopelvic computed tomography showed an approximately 4-cm-sized, soft tissue lesion in the right perianal area. Inguinal lymph node dissection and Mils' operation extended to perianal and perivulvar skin was performed. Histologically, the anal canal lesion was composed of mucin-containing signet ring cells, which were similar to those found in Pagetoid skin lesions. It was diagnosed as an anal canal signet ring cell carcinoma (SRCC) with perianal and vulvar Pagetoid spread and bilateral inguinal lymph node metastasis. Anal canal SRCC is rare, and the current case is the third reported case in the English literature. Seven additional cases were retrieved from the world literature. Here, we describe this rare case of anal canal SRCC with perianal Pagetoid spread and provide a literature review.
Anal Canal* ; Carcinoma, Signet Ring Cell* ; Colonic Polyps ; Female ; Humans ; Lymph Node Excision ; Lymph Nodes ; Middle Aged ; Neoplasm Metastasis ; Paget Disease, Extramammary ; Physical Examination ; Pruritus ; Skin

Here, we report the case of a 43-year-old female who was diagnosed with a cotyledonoid dissecting leiomyoma (CDL) of the uterus. CDL is a recently described and extremely rare variant of a benign leiomyoma that can grossly masquerade as a malignancy. The 13-cm sized tumor was located primarily on the extrauterine surface as an intrauterine continuity, which showed dark red, congested, bulbous protuberances. It was multinodular appearance, encasing the bilateral adnexae and the left iliac vein. Microscopically, the nodules were separated by extensive hydropic degeneration. The nodules were composed of cigar-shaped spindle cells with no mitosis, cellular pleomorphism or coagulation necrosis. They also showed an intravascular luminal growth pattern. CDL with intravascular growth was diagnosed after excluding intravascular leiomyomatosis, disseminated peritoneal leiomyomatosis, and benign metastasizing leiomyoma. The present case is the second reported case of CDL in Korea. Recognition of this rare and bizarre, malignancy-mimicking leiomyoma is crucial to prevent inappropriate treatment.
Adult ; Estrogens, Conjugated (USP) ; Female ; Humans ; Iliac Vein ; Korea ; Leiomyoma* ; Leiomyomatosis ; Mitosis ; Necrosis ; Phenobarbital* ; Uterus*

A 53-year-old woman was presented with several 0.3-0.6 cm-sized nodules within the right lobe of the thyroid. Histologic sections of the thyroid demonstrated multiple papillary microcarcinomas in the background of lymphocytic thyroiditis, with a small focus of Langerhans cell histiocytosis (LCH). Small LCH nodules were also found in the draining cervical lymph nodes. Although the association of LCH with papillary thyroid carcinoma in the thyroid has been reported, their co-existence with LCH in the draining lymph nodes is very rare.
Carcinoma ; Female ; Histiocytosis, Langerhans-Cell ; Humans ; Lymph Nodes ; Middle Aged ; Thyroid Gland ; Thyroid Neoplasms ; Thyroiditis, Autoimmune

Composite pheochromocytoma-ganglioneuroma is one of the mixed neuroendocrine-neural tumors composed of pheochromocytoma and other neural crest derivatives. To date, less than 50 cases of composite pheochromocytoma have been reported, and about 70% of the accompanying tumors were ganglioneuromas. Here, we describe six cases of composite pheochromocytoma-ganglioneuromas in five men and one woman, aged 33 to 64. The size of the tumors ranged from 3.0 to 11.0 cm, and four out of the six presented with intermittent onset of hypertension, palpitation, or dizziness. Microscopically, each tumor was composed of large pleomorphic shaped chromaffin cells arranged in the Zellballen patterns characteristic of pheochromocytoma, and they were mixed with clusters of mature ganglion cells and bundles of spindle-shaped Schwann cells characteristic of ganglioneuroma of variable proportions. All were successfully treated laparoscopically, and none were associated with multiple endocrine neoplasm syndrome or neurofibromatosis. Preoperative diagnosis of a composite pheochromocytoma-ganglioneuroma is impossible because of the low incidence rate, and the radiological findings and symptoms are similar to those of typical pheochromocytomas. Although the significance of microscopic detection of the nonpheochromocytoma component from pheochromocytoma has not yet been clarified, microscopic identification of the composite pheochromocytoma-ganglioneuroma is important because cumulative cases are used in an effort to predict the behavior of this composite tumor.
Adrenal Glands ; Aged ; Chromaffin Cells ; Dizziness ; Female ; Ganglion Cysts ; Ganglioneuroma ; Humans ; Hypertension ; Incidence ; Male ; Neural Crest ; Pheochromocytoma ; Schwann Cells

BACKGROUND: There are few studies of how to diagnose small cell lung cancer in cytological tests through morphometric analysis. We tried to measure and analyze characteristics of small cell carcinoma in lung by image analysis. METHODS: We studied three types of cytologic specimens from 89 patients who were diagnosed with small cell lung cancer by immunohistochemistry. We measured area, perimeter, maximal length and maximal width of cells from small cell carcinoma using image analysis. RESULTS: In lung aspirates, the nuclear mean area, perimeter, maximal length and maximal width of small cell lung cancer were 218.69 microm2, 55 microm, 18.48 microm and 14.65 microm. In bronchial washings, nuclear measurements were 194.66 microm2, 50.07 microm, 16.27 microm and 14.1 microm. In pleural fluid, values were 177.85 microm2, 48.09 microm, 15.7 microm and 13.37 microm. CONCLUSIONS: Nuclear size of small cell lung carcinoma is variable and depends on the cytology method. Nuclei are spindle-shaped and larger in small cell carcinoma from lung aspirates than in bronchial washings or pleural fluid. The cytoplasms of the cells in bronchial washings and pleural fluid were swollen. Therefore, one should consider morphologic changes when trying to diagnose small cell lung cancer through cytological tests.
Bronchoalveolar Lavage ; Carcinoma, Small Cell ; Cytoplasm ; Humans ; Immunohistochemistry ; Lung ; Respiratory Aspiration ; Small Cell Lung Carcinoma

A 62-yr-old man presented with a 5-yr history of intermittent abdominal distention and pain. These symptoms persisted for several months and subsided without treatment. A diagnosis of suspected small bowel lymphoma was made based on plain radiograph and computerized tomogram findings, and he was referred to our institution for further evaluation. Segmental resection of the small intestine was performed and the diagnosis of marginal zone B-cell lymphoma associated with amyloidosis was made. This is the first case of marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) in the small intestine associated with amyloidosis in Korea.
Abdominal Pain ; Amyloidosis/complications/*pathology ; Biopsy ; Diagnosis, Differential ; Endoscopy ; Humans ; Inflammatory Bowel Diseases/diagnosis ; Intestine, Small/*pathology ; Lymphoma, B-Cell, Marginal Zone/complications/*pathology ; Male ; Middle Aged

A 51-yr-old man presented with an enlarged right testis for two months. The radically resected testis showed a relatively well-circumscribed ovoid mass, nearly replacing the normal architecture with central cystic changes. Microscopically, the mass was composed of ovoid shaped tumor cells of a moderately differentiated squamous cell carcinoma (SCC). The central portion of the mass was filled with well-formed laminated keratinous materials and the remnant cavity lined by dysplastic squamous epithelium, indicated SCC may be derived from an epidermal cyst. SCC is among the most common types of neoplasm afflicting human beings, but it is rare in the testis. To our knowledge, this is the second report of the testicular squamous cell carcinoma occurring in a patient without other primary tumors, and the firstly reported case in Korea.
Carcinoma, Squamous Cell/diagnosis/*pathology ; Humans ; Korea ; Male ; Middle Aged ; Testicular Neoplasms/diagnosis/*pathology