A Case of Myxoid Liposarcoma of Neck / 대한두경부종양학회지

Liposarcoma is a very rare malignant tumor affecting the head and neck area. Since it is an invasive and life-threatening disease, active treatments is necessary. However, differential it from lipoma, its corresponding benign counterpart, may be difficult. Herein, we report a rare case of posterior neck myxoid liposarcoma in a 72-year-old man who was initially misdiagnosed with lipoma, including a literature review of differential diagnosis between lipoma and liposarcoma.

Investigation and Standardization on Current Practice of Renal Transplant Pathology in Korea / 대한이식학회지

We need to establish an informative guideline to increase inter-institutional and inter-observer reproducibility of renal transplant diagnosis, and to improve the diagnostic ability of pathologists in Korea. A first nation-wide survey for renal transplant pathology was conducted by Renal Pathology Study Group of the Korean Society of Pathologists in 2016, to provide the continued excellence in the transplantation pathology laboratory, and to improve the diagnostic ability for the best treatment of transplant patients. This survey revealed the significant variations in scale, work load and biopsy indications for the renal transplant pathology in various institutions in Korea. The Banff classification were used by all institutions for the diagnosis of renal transplant pathology, but different formats were used: most institutions (70%) used the “2013 Banff classification” while the others were using “2007 Banff classification” (20%) or even older formats. In daily diagnostic practice of the renal allografts, difficulties that pathologists encounter were quite diverse due to different environments they work in. Most respondents agreed that standardized diagnostic practice guidelines, regular education on renal transplant pathology and convenient ways of consultation are further needed. We are currently working toward the enhancement of the expertise of renal pathologists and to increase inter-institutional and inter-observer reproducibility by 1) development of a set of virtual slides of renal allograft biopsies for the training, 2) validation and gathering expert's consensus on the core variables of rejection diagnosis by using virtual slides, and 3) continued education by the developed virtual slide atlas.

A Case of Polymyositis Associated with Immunoglobulin A Nephropathy

Polymyositis (PM) is a chronic inflammatory disease that predominantly affects muscles. Systemic organ involvement, including the respiratory and gastrointestinal tracts, is frequently observed in PM, but renal involvement is rare. Herein, we report the case of a 56-year-old woman presenting with weight gain, edema, and generalized myalgia. Laboratory tests revealed elevated creatinine kinase level, hypoalbuminemia, and proteinuria. Histopathological examination of muscle biopsy revealed inflammatory myositis, and a renal biopsy confirmed immunoglobulin A (IgA) nephropathy. Based on the clinico-pathological results, the patient was diagnosed with PM with IgA nephropathy. This is a report of a rare occurrence of IgA nephropathy in a patient with PM presenting with chronic glomerulonephritis.

A Case of Bilateral Orbital Tuberculosis

PURPOSE: Periocular tuberculosis is common in the form of conjunctival tuberculosis or uveitis; however, orbital tuberculosis is very rare. We report a case of tuberculosis diagnosed based on bilateral orbital masses. CASE SUMMARY: A 64-year-old male with a history of diabetes, hypertension, and chronic obstructive pulmonary disease presented with diplopia. His best-corrected visual acuity was 1.0, and intraocular pressure was 16 mmHg in both eyes. Bilateral proptosis was noted, and inferior orbital masses were palpated in both eyes. On orbital computed tomography, irregular masses were surrounding both globes in the extraconal space. Incisional biopsy of the bilateral orbital masses was performed under local anesthesia. Pathologic examination revealed chronic granulomatous inflammation accompanied by caseous necrosis. The patient was diagnosed with bilateral orbital tuberculosis and underwent triple therapy with isoniazid, rifampin, and ethambutol for 12 months. The orbital masses completely resolved, and there was no recurrence or complication through 12 months after treatment. CONCLUSIONS: To our knowledge, bilateral orbital tuberculosis has not been reported yet. Herein, we report a case of orbital tuberculosis presenting as bilateral orbital masses.

Effect of low-dose valsartan on proteinuria in normotensive immunoglobulin A nephropathy with minimal proteinuria: a randomized trial

BACKGROUND/AIMS: Immunoglobulin A nephropathy (IgAN) is a generally progressive disease, even in patients with favorable prognostic features. In this study, we aimed to investigate the antiproteinuric effect and tolerability of low-dose valsartan (an angiotensin II receptor blocker) therapy in normotensive IgAN patients with minimal proteinuria of less than 0.5 to 1.0 g/day. METHODS: Normotensive IgAN patients, who had persistent proteinuria with a spot urine protein-to-creatinine ratio of 0.3 to 1.0 mg/mg creatinine, were recruited from five hospitals and randomly assigned to either 40 mg of valsartan as the low-dose group or 80 mg of valsartan as the regular-dose group. Clinical and laboratory data were collected at baseline, and at 4, 8, 12, and 24 weeks after valsartan therapy. RESULTS: Forty-three patients (low-dose group, n = 23; regular-dose group, n = 20) were enrolled in the study. Proteinuria decreased significantly not only in the regular-dose group but also in the low-dose group. The change in urine protein-to-creatinine ratio at week 24 was -41.3% +/- 26.1% (p < 0.001) in the regular-dose group and -21.1% +/- 45.1% (p = 0.005) in the low-dose group. In the low-dose group, blood pressure was constant throughout the study period, and there was no symptomatic hypotension. In the regular-dose group, blood pressure decreased at weeks 8 and 12. No significant change in glomerular filtration rate, serum creatinine level, or serum potassium level was observed during the study period. CONCLUSIONS: Our results suggest that low-dose valsartan can significantly reduce proteinuria without causing any intolerability in normotensive IgAN patients with minimal proteinuria.

High-Grade Urothelial Carcinoma of the Bladder in a Child

Bladder urothelial carcinoma typically occurs in individuals in their sixties or seventies: it rarely occurs in persons <20 years old. Moreover, in young patients, bladder urothelial carcinoma lesions are reported to be solitary and nonmuscle invasive, with low malignant potential. However, 13% of lesions in this age group are reported to be noninvasive high-grade papillary urothelial carcinoma but these are extremely rare in children <15 years. There is no specific consensus regarding treatment of bladder urothelial carcinoma in children and adolescent. In particular, method has been suggested for follow-up of high-grade bladder urothelial carcinoma. Therefore we report our experience of one case the, discuss surveillance methods, and provide a brief review of the literature.

A Case of Thymic Carcinoma with Behcet's Disease Combined with Immunoglobulin A Nephropathy

Behcet's disease is a systemic inflammatory disorder of unknown etiology, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Renal involvement is rare in patients with Behcet's disease particularly immunoglobulin A (IgA) nephropathy. Other autoimmune diseases have been associated with increased risk of malignancy, but not Behcet's disease. Some cases of Behcet's disease accompanied by bladder cancer, thyroid cancer, stomach cancer, or hematologic malignancies have been reported. However, to the best of our knowledge, co-occurrence of Behcet's diseases with thymic carcinoma has not yet been reported. We experienced a 49-year-old male patient who had been treated for Behcet disease and IgA nephropathy, who presented with a large mediastinal mass on chest x-ray. After thymectomy, he was diagnosed with thymic carcinoma with complete resection.

Solitary Fibrous Tumor of Central Nervous System: A Case Report

Solitary fibrous tumor (SFT) is a rare neoplasm of mesenchymal origin, especially in the central nervous system (CNS). Reported herein is a case of SFT of CNS in a 63-year-old female patient who had confused mentality, without other neurological deficit. The brain MRI showed an ovoid mass in the right frontal lobe. The tumor was surgically removed grossly and totally, and the pathologic diagnosis was SFT. At 55 months after the surgery, the tumor recurred at the primary site and at an adjacent area. A second operation was thus done, and the tumor was again surgically removed grossly and totally. The pathologic diagnosis was the same as the previous, but the Ki-67 index was elevated. Ten months later, two small recurring tumors in the right frontal skull base were found in the follow-up MRI. It was decided that radiation therapy be done, and MRI was done again 3 months later. In the follow-up MRI, the size of the recurring mass was found to have decreased, and the patient did not manifest any significant symptom. Follow-up will again be done 18 months after the second surgery.

The Olfactory Groove Schwannoma Attached to the Cribriform Plate: A Case Report

The olfactory groove schwannoma is a quite rare tumor. We report a case of a 49-year-old woman with an olfactory groove schwannoma attached to the cribriform plate without olfactory dysfunction. She had no specific neurological symptoms other than a headache, and resection of the tumor showed it to be a schwannoma. About 19 months after the operation, a follow-up MRI showed no evidence of tumor recurrence. Surgical resection through subfrontal approach could be one of the curative modality in managing an olfactory groove schwannoma. An olfactory groove schwannoma should be considered in the differential diagnosis of anterior skull base tumors.

Solitary Ancient Schwannoma in Upper Arm: A Case Report / 대한골관절종양학회지

Ancient schwannoma is a variant of schwannoma and is characterized slowly growing tumor with degenerative change. And it is reported that schwannoma is relatively rare in extensor area. As a rare cause of solitary ancient schwannoma in extensor area of upper arm, we report it.

A Case of Primary Eyelid Peripheral T-Cell Lymphoma, Not Otherwise Specified

PURPOSE: To report a case of primary peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), of the eyelid. METHODS: A 48-year-old healthy male patient presented with a mass on the upper lid of 2 months in duration. The lesion was reddish, well-demarcated, oval shaped, and measured approximately 8 x 4 mm. The mass did not respond to incisional drainage and intra-lesional triamcinolone. An excisional biopsy for diagnosis was performed. RESULTS: On microscopic examination, a localized dense lymphocytic infiltration was observed in the subepithelial area, and cytologic atypia was observed under high power. On immunohistochemical examination, tumor cells were positive for CD3 but negative for CD20, CD30, CD56, k-light chain immunoglobulin, lambda-light chain immunoglobulin, and increased Ki-67 activity was noted. A histopathological diagnosis of PTCL-NOS was made. CONCLUSIONS: PTCL-NOS, which rarely occurs on the eyelids, commonly accompanies generalized lymphadenopathy and "B symptoms" such as fever and weight loss. Herein, the authors report a case of PTCL-NOS of the eyelid presenting as a rapidly growing solid mass in an otherwise healthy patient.

Immunoglobulin A Nephropathy Associated with Plasmodium falciparum Malaria

Glomerulonephritis occurs as a rare form of renal manifestation in Plasmodium falciparum malaria. Herein, we report a case of falciparum malaria-associated IgA nephropathy for the first time. A 49-yr old male who had been to East Africa was diagnosed with Plasmodium falciparum malaria. Microhematuria and proteinuria along with acute kidney injury developed during the course of the disease. Kidney biopsy showed mesangial proliferation and IgA deposits with tubulointerstitial inflammation. Laboratory tests after recovery from malaria showed disappearance of urinary abnormalities and normalization of kidney function. Our findings suggest that malaria infection might be associated with IgA nephropathy.

A Case of Ankylosing Spondylitis with Follicular Lymphoma

Several autoimmune and chronic inflammatory conditions have been consistently linked with an increased risk of hematologic malignancies. Although ankylosing spondylitis (AS) is a chronic inflammatory disease, previous studies have demonstrated that it is not associated with an increase in risk of malignant lymphomas. Cases of AS accompanied by hematologic malignancies such as multiple myeloma, chronic myelogenous leukemia, and Hodgkin's disease have been reported. In Korea, AS with non-Hodgkin's lymphoma or follicular lymphoma has not been reported. We experienced a 38-year-old male who had been diagnosed with follicular lymphoma with bone metastasis, who achieved complete remission after having been treated with chemotherapy, developed new inflammatory back pain. An MRI of his hip showed an active inflammation of the left sacroiliac joint and a positive HLA-B27. The patient was diagnosed with AS and was treated with naproxen, which improved the pain in his back and buttock.

A Case of AA Amyloidosis Treated with Infliximab / 대한신장학회지

AA amyloidosis is one of the most significant complications of rheumatoid arthritis characterized by the deposition of amyloid A (AA) in multiple organs and tissues in the body. This disorder displays variable clinical symptoms depending upon the involved organ and a diagnosis is rendered through a biopsy of the affected organ, followed by staining using Congo-red which reveals an apple-green birefringence. Fundamental disease control is critical in the treatment of AA amyloidosis. Anti-tumor necrosis factor alpha (anti TNF-alpha) agents are promising in inducing clinical remission by suppressing systemic inflammation in AA amyloidosis. We report a case of AA amyloidosis in a 71 year old woman with rheumatoid arthritis that responded well to infliximab therapy.

A Case of IgA Nephropathy associated with Acute Hepatitis A / 대한신장학회지

IgA nephropathy is the most common type of glomerulonephritis worldwide. Although primary IgA nephropathy receives the most attention, many other diseases are also associated with IgA nephropathy. Among these, chronic liver diseases such as alcoholic liver disease or hepatitis B or C have been reported as secondary causes of glomerular IgA deposits. Recently, as the prevalence of hepatitis A virus (HAV) infection is increasing in Korea, HAV-associated renal diseases occur frequently. Acute kidney injury (AKI) is one of the most common complications of HAV infection, mainly due to acute tubular necrosis or interstitial nephritis. However, unlike hepatitis B and C, glomerular involvement is extremely rare in acute HAV infection. Here, we report a case of biopsy-proven IgA nephropathy with serologically documented HAV infection. The patient presented moderate degree of proteinuria without evidence of AKI. Renal biopsy revealed mesangial IgA deposits but tubular or interstitial inflammation was not observed. This report suggests that HAV infection may be a secondary cause of IgA nephropathy. However, further studies are required to elucidate a causal link between hepatitis A and mesangial IgA deposits.

Spontaneous Remission of Nephrotic Syndrome in IgA Nephropathy / 대한신장학회지

Although a moderate degree of proteinuria is common in patients with IgA nephropathy (IgAN), nephrotic syndrome combined with IgAN is rare. It has been reported that approximately 5% of all patients with IgAN are complicated by minimal change disease and these patients respond well to corticosteroid therapy. However, spontaneous remission is uncommon in heavy proteinuric patients with IgAN. Recently, we experienced two cases of spontaneous remission of nephrotic syndrome combined with IgAN without use of corticosteroid. In these patients, heavy proteinuria disappeared in 1 month after the onset and no recurrence occurred during follow-up. With such limited number of cases, factors associated with spontaneous remission in these patients could not be explored. Further study with a larger number of patients is required and careful attention should be paid to these complicated cases.

Intravascular Papillary Endothelial Hyperplasia (Masson's Tumor) of the Chest Wall: A Case Report

Intravascular papillary endothelial hyperplasia is a rare tumor-like lesion caused by hyperplastic proliferation of endothlial cells that is usually an incidental findings within thrombosed dilated blood vessels or vascular tumor. We report the sonographic appearance and pathological correlation of intravascular papillary endothelial hyperplasia that presented as an intramuscular solitary mass in chest wall.

A Case of Pleural Metastasis from Papillary Tthyroid Carcinoma / 결핵및호흡기질환

Lung cancer, breast cancer and lymphoma are the common oncologic causes of malignant pleural effusion, comprising more than the half of the causes. However, an endocrinologic carcinoma associated malignant effusion is very rare. Recently, we encountered a case of papillary thyroid carcinoma causing malignant effusion. An 83-year-old female patient presented with dyspnea due to massive pleural effusion in her left side. The pleural biopsy, pleural fluid cytology and breast needle aspiration biopsy results were consistent with a metastatic papillary thyroid carcinoma. Thyroid ultrasonography showed two thyroid masses, but the patient refused a thyroid biopsy. This case highlights the need for considering the possibility of papillary thyroid carcinoma when the cause of malignant pleural effusion cannot be found because one of the rare clinical manifestations of a papillary thyroid carcinoma can be dyspnea due to malignant effusion.

A Case of Pleural Metastasis from Papillary Tthyroid Carcinoma / 결핵및호흡기질환

Lung cancer, breast cancer and lymphoma are the common oncologic causes of malignant pleural effusion, comprising more than the half of the causes. However, an endocrinologic carcinoma associated malignant effusion is very rare. Recently, we encountered a case of papillary thyroid carcinoma causing malignant effusion. An 83-year-old female patient presented with dyspnea due to massive pleural effusion in her left side. The pleural biopsy, pleural fluid cytology and breast needle aspiration biopsy results were consistent with a metastatic papillary thyroid carcinoma. Thyroid ultrasonography showed two thyroid masses, but the patient refused a thyroid biopsy. This case highlights the need for considering the possibility of papillary thyroid carcinoma when the cause of malignant pleural effusion cannot be found because one of the rare clinical manifestations of a papillary thyroid carcinoma can be dyspnea due to malignant effusion.

A Case of Bronchiolitis Obliterans Organizing Pneumonia from Epstein-Barr Virus / 결핵및호흡기질환

In the average adult with a normal immune state, Epstein-Barr virus pneumonia is very rare, especially in the form of interstitial lung disease. According to recent studies, the Epstein-Barr virus is also associated with lymphocytic interstitial pneumonia, AIDS and Langerhans cell histiocytosis, but not with sarcoidosis. BOOP is caused by lung injury due to an infection or drug intoxication, and is related to connective tissue disease or bone marrow transplantation, but is sometimes idiopathic. We experienced a patient with symptoms and signs of interstitial lung disease, with confirmed BOOP and EBV ingection from an open lung biopsy and serologic examination, respectively Herein, this case is reported, with a review of the literature.