Chondroid syringoma is a rare mixed tumor of the skin which is composed of both mesenchymal and epithelial cells. Its incidence at less than 0.1% and is frequently located on the head and neck. Chondroid syringoma is easily confused with epidermal cysts. Since malignant forms of chondroid syringoma have been reported, accurate and timely diagnosis is important for proper management. We report clinical and histological features of chondroid syringoma in 5 patients treated at our institution. In most of the cases, chondroid syringoma presented as a round, firm, nodular or cystic lesion that had well marginated heterogeneity in sonography. Clinically, all of the lesions were removed by simple excision. Microscopically, all five tumors were well circumscribed and consisted of epithelial, myoepithelial, and stromal components. The epithelial component formed tubules lined by one or more rows of eosinophilic epithelial cells. The outer layer of tubules appeared to be flattened myoepithelial cells. The stroma is myxoid and contained spindle shaped myoepithelial cells. We expect that the clinical, sonographic, and histological data from our report may help clinicians who are confronted with various kinds of analogous facial lesions to decide the most proper management for their patients.

Abstract Glomangiomatosis, a rare category of glomus tumors, comprises 2% to 3% of glomus tumors in adults. We report a case of glomangiomatosis in a 48-year-old man who underwent multiple excisions of recurrent soft tissue masses in the ankle and foot. Ultrasonography revealed multiple nodular soft tissue tumors and dilated vascular channels with increased blood flow, suggesting the presence of vascular tumors. Here, we discuss the ultrasonography imaging features of glomangiomatosis, which have not been previously reported, correlate them with magnetic resonance imaging findings, and provide a brief review of the literature.

Glomangiomatosis, a rare category of glomus tumors, comprises 2% to 3% of glomus tumors in adults. We report a case of glomangiomatosis in a 48-year-old man who underwent multiple excisions of recurrent soft tissue masses in the ankle and foot. Ultrasonography revealed multiple nodular soft tissue tumors and dilated vascular channels with increased blood flow, suggesting the presence of vascular tumors. Here, we discuss the ultrasonography imaging features of glomangiomatosis, which have not been previously reported, correlate them with magnetic resonance imaging findings, and provide a brief review of the literature.

Malignant lymphoma of the upper urinary tract including the renal pelvis and ureter is extremely rare. Less than ten cases have been reported in the literature, of which extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is the most frequent histologic type [1]. MALT lymphoma arising in the stomach, skin, thyroid, and salivary gland is associated with chronic inflammation caused by pathogenic microorganisms or autoimmune disorder [2]; however, the pathogenesis of MALT lymphoma in the upper urinary tract is unknown. Herein, we report a case of upper ureteral MALT lymphoma with extensive peri-ureteropelvic inflammation and atherosclerosis of the renal artery, and discuss the possible correlation between these lesions.

An inflammatory pseudotumor (IPT) is a rare tumor composed of proliferative myofibroblasts, fibroblasts, histiocytes, plasma cells, and lymphocytes (1). IPT is mainly seen in young patients and often involves the lung and orbit, but it may occur in virtually any anatomic location and in patients of any age. Although IPT usually presents as an isolated mass in a single organ, it sometimes manifests in the form of multifocal lesions that are concentrated in one anatomic region (2). Few IPT cases have been reported in the ureter and bladder. The lesions are usually benign, but some abdominal cases have exhibited malignant potential or metastasis. Recently, some experts have suggested classifying IPT into three categories, including plasma cell-rich IPT [also known as lymphoplasmacytic or immunoglobulin G4 (IgG4)-related IPT] (3). In this case report, we describe the clinical, imaging, and pathology lab results of an IgG4-related IPT that occurred in a patient’s periureteral area, paravesical space, and prostate.

An inflammatory pseudotumor (IPT) is a rare tumor composed of proliferative myofibroblasts, fibroblasts, histiocytes, plasma cells, and lymphocytes (1). IPT is mainly seen in young patients and often involves the lung and orbit, but it may occur in virtually any anatomic location and in patients of any age. Although IPT usually presents as an isolated mass in a single organ, it sometimes manifests in the form of multifocal lesions that are concentrated in one anatomic region (2). Few IPT cases have been reported in the ureter and bladder. The lesions are usually benign, but some abdominal cases have exhibited malignant potential or metastasis. Recently, some experts have suggested classifying IPT into three categories, including plasma cell-rich IPT [also known as lymphoplasmacytic or immunoglobulin G4 (IgG4)-related IPT] (3). In this case report, we describe the clinical, imaging, and pathology lab results of an IgG4-related IPT that occurred in a patient’s periureteral area, paravesical space, and prostate.

To the Editor, Sarcoidosis is a systemic granulomatous disorder of unclear etiology that affects the lungs, eyes, skin, heart, and other organs [1]. Subcutaneous sarcoidosis occurs less frequently and is seen in approximately 12.2% of Asian patients with sarcoidosis [2]. Subcutaneous sarcoidosis is characterized by an insidious onset of multiple small subcutaneous nodules that are commonly located on the extremities (78.8%), trunk, face, and groin [3]. Although subcutaneous sarcoidosis is strongly associated with systemic disease, severe hypercalcemia, and acute kidney injury (AKI) are rarely reported [3,4]. We herein report a case of subcutaneous sarcoidosis presenting as a large, fixed suprapubic mass mimicking malignancy. The case was complicated by severe hypercalcemia and AKI, and it did not involve the lungs. A 61-year-old Korean man presented to the hospital with a progressively enlarging suprapubic mass. He had been diagnosed with diabetes mellitus and hypertension 10 years previously. Two years before admission, he was diagnosed with membranous nephropathy and treated with cyclosporine and prednisolone. At that time, his creatinine (Cr) level was 1.53 mg/dL. A physical examination revealed a firm, nontender, round, fixed mass without skin lesions in the suprapubic area. There was no palpable lymphadenopathy or hepatosplenomegaly. Initial laboratory findings showed serum Cr and serum calcium (Ca) levels of 2.0 and 11.0 mg/dL, respectively. After 12 days, the serum Cr and Ca levels increased to 6.2 and 14.7 mg/dL, respectively. Other laboratory results were as follows: serum level of blood urea nitrogen, 55.9 mg/dL; ionized Ca, 1.91 mmol/L (reference range, 1.13 to 1.32); phosphorous, 4.5 mg/dL; intact parathyroid hormone, 8.1 pg/mL (reference range, 14 to 72); serum alkaline phosphatase, 47 IU/L (reference range, 42 to 128); 1,25-dihydroxyvitamin D, 71.2 pg/mL (reference range, 25.1 to 66.1); parathyroid hormone related peptide, < 1.1 pmol/L; angiotensin-converting enzyme, 110.2 U/mL (reference range, 9 to 47); thyroid-stimulating hormone, 0.706 µIU/mL; hemoglobin, 12.1 g/dL; white blood cell count, 9.8 × 103/µL; platelet count, 370 × 103/µL; urine Ca/Cr ratio, 303 mg/g; and 24-hour urine protein, 312.8 mg/day. Abdominal computed tomography (CT) revealed a 4.4 × 3.3-cm suprapubic mass in the subcutaneous area (Fig. 1A). A chest CT scan showed did not show hilar lymphadenopathy (Fig. 1B). 18F-fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) showed multiple foci of hypermetabolic activity in the suprapubic area, both shoulders, flank, hip, and thigh, and there were no hypermetabolic lesions in the lung (Fig. 1C and 1E). He was treated with intravenous fluids, furosemide, bisphosphonate, calcitonin, and renal replacement therapy (continuous venovenous hemodialysis). However, his Ca and Cr levels were persistently elevated at 11.0 and 5.1 mg/dL, respectively. The suprapubic mass was excised and histologically identified as noncaseating granulomatous inflammation within fat tissue. The solid granuloma was composed of epithelioid histiocytes and multinucleated giant cells engulfing fat vacuoles and mild lymphocytic infiltration (Fig. 2). The patient was started on oral prednisolone 30 mg/day, and his Ca level decreased to 9.6 mg/dL after 1 week of treatment. After 2 weeks, his Cr level improved to 2.3 mg/dL (Fig. 3). Prednisolone was slowly tapered off over 3 months. After 3 months of treatment, a follow-up 18F-FDG PET/CT scan demonstrated complete resolution of the multiple hypermetabolic lesions (Fig. 1D and 1F), and his Cr and Ca levels were 2.0 and 8.8 mg/dL, respectively. His renal function and serum Ca levels remain stable after 1 year of treatment. The subcutaneous sarcoidosis associated with hypercalcemia and AKI was successfully treated with a moderate dose of prednisolone. Figure 1 Imaging findings in a patient with subcutaneous sarcoidosis. (A) Abdominal computed tomography (CT) scan showing a 4.4 × 3.3-cm suprapubic mass in the subcutaneous area (arrow). (B) Chest CT scan showing lobulated, calcified nodules in the left ... Figure 2 Histopathology of the subcutaneous mass. Solid conglomerated granulomas composed of multiple small granulomas within the subcutaneous fat tissue. The small granulomas are composed of epithelioid cells, multinucleated giant cells, and minimal lymphocytic ... Figure 3 Patient's serum creatinine and calcium levels plotted against time for 2 months following presentation. The serum creatinine and calcium remarkably decreased after the prednisolone treatment. CVVHD, continuous venovenous hemodialysis. Subcutaneous sarcoidosis is observed in 2% to 12% of patients with systemic sarcoidosis [2,3]. It presents with insidious onset of multiple, nontender, firm, mobile, and round subcutaneous nodules. The lesions vary from 1 to 100 in number and from 0.5 to 2.0 cm in size; the skin lesions are usually asymptomatic. The lesions are mainly distributed in the extremities (76% to 100%), followed by the trunk (28%), buttocks (10%), and forehead (5%) [3]. Subcutaneous sarcoidosis is frequently associated with systemic diseases such as bilateral hilar lymphadenopathy (94%), arthritis (19%), peripheral neuropathy (13%), renal involvement (13%), and uveitis (13%) [3]. In this case, the patient presented with a single large mass in the suprapubic area. There was no bilateral hilar lymphadenopathy or palpable lesion other than the suprapubic mass. Additionally, the patient showed severe hypercalcemia and AKI, which is a rare presentation of subcutaneous sarcoidosis [4]. Malignancies such as lymphoma and soft tissue sarcoma were considered as differential diagnoses. The diagnosis of subcutaneous sarcoidosis was based on the patient's clinical and radiological findings and supported by the histopathological findings of a noncaseating epithelioid granuloma predominantly involving panniculus with minimal inflammation and without evidence of organisms or exogenous particles [1,3]. The sarcoidosis was characterized by infiltration of CD4 type 1 helper cells and macrophages into the sites of inflammation, and these cells increased the 18F-FDG uptake [5]. 18F-FDG PET/CT scans showed good sensitivity (87%) in detecting all biopsy-proven sarcoidosis localizations excluding skin involvement [5]. 18F-FDG PET/CT revealed multiple sites of involvement in the subcutaneous and muscle layers at the time of diagnosis, and complete resolution of the lesions was seen after the prednisolone treatment. 18F-FDG PET/CT was useful in evaluating the extent of disease and follow-up response to therapy in this patient. Hypercalciuria is the most common abnormality of Ca metabolism in sarcoidosis, ranging from 40% to 50% [1]. Although hypercalcemia occurs in 7.4% to 11.0% of patients, clinically significant hypercalcemia is less common, with a prevalence of < 5% of patients [1,5]. However, hypercalciuria was not present despite the severe hypercalcemia in this patient. An increased plasma 1,25-dihydroxyvitamin D level is a probable cause of hypercalcemia, and macrophages in patients with sarcoidosis contain 25-hydroxyvitamin D-1α-hydroxylase, which converts 25-hydroxyvitamin D to 1,25-dihydroxyvitamin D [1]. Corticosteroids are the drugs of choice to decrease the endogenous production of 1,25-dihydroxyvitamin D [1,3]. Oral prednisolone at 20 to 40 mg/day has been suggested as the initial treatment dose for sarcoidosis, and this can be reduced over a period of 1 to 3 months [1]. Evidence supporting the use of immunosuppressive agents such as methotrexate is currently lacking. In summary, we have reported a patient with subcutaneous sarcoidosis who presented with a suprapubic mass, hypercalcemia, and AKI and was successfully treated with a moderate dose of prednisolone.

Uterine rhabdomyosarcoma (RMS) typically presents as a mixed epithelial and mesenchymal tumors. Pure RMSs of the female genital tract are uncommon. Spindle cell variant of RMS is a rare morphologic subtype of embryonal RMS and mostly occurs in the paratesticular region of children. Here, we present a case of uterine spindle cell RMS in a 76-year-old woman. The tumor, 20×15×7 cm in size, was highly necrotic and adherent to the colon and rectum. Tumor cells were mostly spindle-shaped, and isolated rhabdomyoblasts were scattered. Immunohistochemical stains for myoglobin and myo-D1 showed diffuse positivity for tumor cells. The patient died only of disease three months after diagnosis.

Background The incidence of early colorectal epithelial neoplasm (ECEN) is increasing, and its pathologic diagnosis is important for patient care. We investigated the incidence of ECEN and the current status of its pathologic diagnosis. Methods We collected datasheets from 25 institutes in Korea for the incidence of colorectal adenoma with high grade dysplasia (HGD) and low grade dysplasia in years 2005, 2007, and 2009; and early colorectal carcinoma in the year 2009. We also surveyed the diagnostic terminology of ECEN currently used by the participating pathologists. Results The average percentage of diagnoses of adenoma HGD was 7.0%, 5.0%, and 3.4% in years 2005, 2007, and 2009, respectively. The range of incidence rates of adenoma HGD across the participating institutes has gradually narrowed over the years 2005 to 2009. The incidence rate of early colorectal carcinoma in the year 2009 was 21.2%. The participants did not share a single criterion or terminology for the diagnosis of adenoma HGD. The majority accepted the diagnostic terms that distinguished noninvasive, mucosal confined, and submucosal invasive carcinoma. Conclusions Further research requirements suggested are a diagnostic consensus for the histopathologic diagnosis of ECEN; and standardization of diagnostic terminology critical for determining the disease code.

Colorectal cancer is rare in teenagers, especially without known risk factors. Colon cancer in young age is more likely to be diagnosed at advanced-stage, to present unfavorable tumor histology such as mucinous carcinoma, and poor outcome. We report a case of sporadic mucinous adenocarcinoma of the colon in a 19-year-old male patient without any risk factors. He complained of severe left abdominal pain that developed 1 month ago. He had a distended abdomen with severe tenderness on the left lower quadrant. A distal descending colon mass causing mechanical obstruction was observed on abdominal computed tomography. Emergency colonoscopy showed a large, fungating mass obstructing the lumen at 40 cm from the anal verge. Biopsy of the colonic mass suggested a mucinous adenocarcinoma. After decompression by colonic stent, the patient was transferred to the general surgery department for left hemicolectomy. The lesion was confirmed to be a mucinous adenocarcinoma (7.0×4.5 cm). For hereditary nonpolyposis colorectal cancer evaluation, immunohistochemical staining for MLH1 and MSH2 was normal. Reverse transcription polymerase chain reaction analysis did not detect microinstability in any of the markers tested. The patient had no familial history of cancer. Mucinous adenocarcinoma has high frequencies of poor differentiation, advanced tumor stage, loss of mismatch repair gene expression, and increased MUC2 expression. A mucinous histology is considerably more frequent in children and adolescent than in adults. Adequate invasive study is also necessary for young age patients.

The histopathological diagnosis of gastric mucosal biopsy and endoscopic mucosal resection/endoscopic submucosal dissection specimens is important, but the diagnostic criteria, terminology, and grading system are not the same in the East and West. A structurally invasive focus is necessary to diagnose carcinoma for most Western pathologists, but Japanese pathologists make a diagnosis of cancer based on severe dysplastic cytologic atypia irrespective of the presence of invasion. Although the Vienna classification was introduced to reduce diagnostic discrepancies, it has been difficult to adopt due to different concepts for gastric epithelial neoplastic lesions. Korean pathologists experience much difficulty making a diagnosis because we are influenced by Japanese pathologists as well as Western medicine. Japan is geographically close to Korea, and academic exchanges are active. Additionally, Korean doctors are familiar with Western style medical terminology. As a result, the terminology, definitions, and diagnostic criteria for gastric intraepithelial neoplasia are very heterogeneous in Korea. To solve this problem, the Gastrointestinal Pathology Study Group of the Korean Society of Pathologists has made an effort and has suggested guidelines for differential diagnosis: (1) a diagnosis of carcinoma is based on invasion; (2) the most important characteristic of low grade dysplasia is the architectural pattern such as regular distribution of crypts without severe branching, budding, or marked glandular crowding; (3) if nuclear pseudostratification occupies more than the basal half of the cryptal cells in three or more adjacent crypts, the lesion is considered high grade dysplasia; (4) if severe cytologic atypia is present, careful inspection for invasive foci is necessary, because the risk for invasion is very high; and (5) other structural or nuclear atypia should be evaluated to make a final decision such as cribriform pattern, papillae, ridges, vesicular nuclei, high nuclear/cytoplasmic ratio, loss of nuclear polarity, thick and irregular nuclear membrane, and nucleoli.

Waldenstrom macroglobulinemia (WM) is a B-cell lymphoproliferative disorder associated with bone marrow involvement of lymphoplasmacytic lymphoma (LPL) and an IgM monoclonal gammopathy. Generally B-lymphocytes in LPL do not express CD5 that is important for differential diagnosis of B-cell lymphoproliferative disorders. In WM, various renal diseases and type I cryoglobulinemia are well described separately, but cryoglobulinemic glomerulonephropathy is very rarely reported. A 61-yr-old woman complained of generalized edema, cyanosis of the extremities in cold weather, visual disturbance, and pancytopenia. Bone marrow and renal biopsy showed CD5+ expressing B-cells and cryoglobulinemic glomerulonephropathy. With the diagnosis of WM, she received cyclophosphamide, doxorubicin, vincristine and prednisolone chemotherapy and got complete remission. Here, we report a rare case of WM associated with unusual expression of CD5+ B-lymphocytes and cryoglobulinemic glomerulonephropathy, and emphasize the importance of the clinical features in differentiating CD5+ B-cell lymphoproliferative disorders.

BACKGROUNDAnnexin A1 (ANXA1) is known to be involved in the progression and differentiation of various tumors. However, its significance and role in bladder carcinogenesis has not been fully elucidated. To determine the role ANXA1 plays in urothelial carcinoma (UC), we investigated the expression of ANXA1 protein in normal urothelial tissue, carcinoma in situ (CIS), and UC of the urinary bladder.METHODSProtein expression level of ANXA1 and its subcellular localization were analyzed in 88 cases of UCs and corresponding 24 normal tissues and 24 CISs by immunohistochemistry.RESULTSANXA1 was significantly down-regulated at all subcellular localization in CIS and in the cytoplasm and membrane of cells of UC, compared to normal tissues. No significant correlation between ANXA1 expression level and tumor depth (pT), growth pattern, and recurrence was found. However, cytoplasmic and membranous ANXA1 were significantly up-regulated in high grade than in low grade UC (p=0.02 in cytoplasm and p=0.03 in membrane).CONCLUSIONSThese results suggest that ANXA1 dysregulation is involved in urothelial carcinogenesis and ANXA1 is potentially a marker for the pathologic differentiation of UC.

Enterobius vermicularis is one of the most common parasites found in the intestine of humans. The gravid female worms migrate outside the anus to release eggs on the perianal skin. Rarely, they migrate to the genitourinary tract in female patients. We present a case in which pinworm eggs were found in a cervicovaginal smear of a 37-year-old woman. The eggs were elongated oval shaped and flattened on one side. The thick, double contoured birefringent shell stained bright yellow or orange. Some coarsely granular embryos or curved larvae were enclosed in the refractile shell. Empty eggs or wrinkled shells with clumped granular material were also present. Although pinworm eggs are easily identified because of their characteristic morphologic appearance, careful screening is needed due to the frequent masking by inflammatory cells.

Background : To standardize renal biopsy reporting and diagnosis, The Renal Pathology Study Group of the Korean Society of Pathologists (RPSKSP) has developed a renal pathology reporting format for the native and allograft kidney. Methods : A consensus checklist of a provisional renal biopsy format was sent to all members of the RPSKSP. Feed back opinions regarding the practical application of the checklist to the diagnostic work were received. Results : Kidney biopsies require three essential examinations: by light microscopy, immunofluorescence (IF), and electron microscopy (EM). A final report of a renal biopsy should include information on specimen adequacy and a description of the morphologic change using a systematic semiquantitative method for each of the compartments, with optional separate IF and EM reports. Conclusions : A standard renal biopsy report format is important in establishing clinicopathologic correlations, making reliable prognostic considerations, comparing the findings in sequential biopsies and evaluating the effects of therapy. This renal report system could encourage nationwide multi-institutional collaborative studies.

Metabolites of colorectal cancer tissues from 12 patients were analyzed and compared with those of the normal tissues by two-dimensional NMR spectroscopy. NMR data were analyzed with the help of the metabolome database and the statistics software. Cancerous tissues showed significantly altered metabolic profiles as compared to the normal tissues. Among such metabolites, the concentrations of taurine, glutamate, choline were notably increased in the cancerous tissues of most patients, and those of glucose, malate, and glycerol were decreased. Changes in individual metabolites varied significantly from patient to patient, but the combination of such changes could be used to distinguish cancerous tissues from normal ones, which could be done by PCA analysis. The traditional chemometric analysis was also performed using AMIX software. By comparing those two results, the analysis via (1)H-(13)C HSQC spectra proved to be more robust and effective in assessing and classifying global metabolic profiles of the colorectal tissues.

Desmoid tumor is a rare benign tumor derived from fibrous sheath or musculoaponeurotic structure. The tumor is benign histologically but considered as malignant clinically because it has high propensity on infiltrative growth with local invasion and tendency to recurrence after local excision. Especially, when this tumor happens to be in the intra-abdomen, the prognosis is worse because it can cause intestinal obstruction, ureter obstruction and, fistula formation. It also can invade major vessels in abdomen. This tumor occurs more frequently in patients with familial adenomatous polyposis (FAP), in post-partume women, and at old surgical incision site. However, in this case, the patient had neither previous surgery nor a FAP history. We report a rare case of the young male patient who presented with an acute abdomen and underwent laparotomy and was found to have an intra-abdominal desmoid tumor with abscess formation.

Sarcoidosis is a granulomatous multisystemic disorder that rarely involves subcutaneous tissue. We describe the MR imaging findings of a subcutaneous sarcoidosis in a patient that presented with a nontender, palpable soft tissue mass on the left buttock, which was confirmed after surgical excision. The MR images showed the presence of a subcutaneous mass that breached the adjacent fascia with an irregular outline and homogeneous, slightly higher signal intensity than the surrounding muscle as seen on a T2-weighted image and with homogeneous enhancement after contrast injection. The lesion could not be differentiated from a sarcoma or a malignancy.

Intrahepatic cholangiocarcinoma is a malignant neoplasm arising from the biliary epithelium, which frequently invades adjacent organs or metastasizes to other visceral organs such as the lungs, bones, adrenals, and brain. However, distant skeletal muscle metastasis of cholangiocarcinoma has never been described before to the best of our knowledge and, furthermore, Budd-Chiari syndrome secondary to intrahepatic cholangiocarcinoma is also extremely rare. Here we present the first case overall of distant muscle metastasis from intrahepatic cholangiocarcinoma presenting as Budd-Chiari syndrome. A 44-year-old man admitted to the hospital with complaints of abdominal distension, edema of both legs, back pain and anorexia of 30 d' duration. Computed tomography and ultrasonography-guided percutaneous muscle biopsy established intrahepatic cholangiocarcinoma with disseminated thrombosis from inferior vena cava to bilateral iliac and femoral veins, and multiple skeletal muscle metastases in bilateral buttock and erector spinal muscle.