A Rare Occurrence of Primarily Extranodal Spinal Epidural Lymphoma With Spinal Cord Compression and Invasion to the Thoracic Cavity

A 41-year-old man suffered from progressive radiculomyelopathy caused by spinal epidural mass primarily encasing the spinal cord at the cervicothoracic vertebrae that extended into the thoracic cavity through the neural foramen. An urgent decompressive laminectomy and epidural tumor resection were performed to prevent neurological deterioration and effective spinal cord decompression. The histopathologic diagnosis was diffuse large B-cell lymphoma. As first-line treatment for stage II extranodal lymphoma, he received 6 cycles of R-CHOP (rituximab/cyclophosphamide, hydroxydaunorubicin, Oncovin, and prednisone) chemotherapy. Consequently, follow-up positron-emission tomography CT and MR images demonstrated a complete metabolic response (Deauville score 1). This rare occurrence of primarily extranodal spinal epidural lymphoma with limited disease will be presented in a literature review.

Effect of Severe Limb Purpura Following the Administration of COVID-19Vaccination on a Diabetic Foot Requiring Amputation: A Case Report / 대한족부족관절학회지

The current SARS-CoV‑2 coronavirus disease 2019 (COVID-19) pandemic has been a particular challenge for diabetes patients. Since these patients are at a higher risk of COVID-19, they have been prioritized for vaccination. In this report, we describe the case of a patient scheduled for diabetic foot amputation who received the first dose of ChAdOx1 nCov-19 vaccine and subsequently developed severe purpura in his genitalia and both of his hands and feet, accompanied by acute renal failure. The operation had to be postponed as severe limb purpura appeared just before the operation. With adequate management for acute renal failure and topical steroid application for the severe purpura lesions, a successful outcome could be obtained after the delayed first ray amputation. We recommend that COVID-19 vaccination should be carefully administered in patients with a diabetic foot requiring amputation.

Diffuse Involvement of Primary Colorectal Lymphoma Simulating Ulcerative Colitis

Diffuse involvement of colorectal lymphoma masquerading as colitis is a very rare presentation of primary colorectal lymphoma. Detecting occult lymphoma is difficult in the setting of diffuse colonic involvement with no definite mass and inflammatory mucosal changes. We encountered a case of diffuse-type primary colorectal lymphoma simulating ulcerative colitis in a previously healthy 31-year-old woman. Despite multiple mucosal biopsies, the biopsy diagnosis was not made due to unawareness of atypical lymphocytes admixed with dense lymphoplasmacytic infiltration. The present case emphasizes the importance of being aware of this rare presentation of primary colorectal lymphoma in order to avoid misdiagnosis.

Provisional Guideline Recommendation for EGFR Gene Mutation Testing in Liquid Samples of Lung Cancer Patients: A Proposal by the Korean Cardiopulmonary Pathology Study Group

Liquid biopsy for detection of mutation from circulating tumor DNA is a new technology which is attractive in that it is non-invasive. Epidermal growth factor receptor (EGFR) tyrosine kinase inhibitors (TKI) is an effective first line drug for advanced non-small cell lung cancer patients who harbor activating EGFR mutation. During the course of treatment, resistance against TKI arises which can be contributed to EGFR T790M mutation in about 50–60% of patients. Third generation TKI may overcome the resistance. In patients who cannot undergo tissue biopsy due to variable reasons, liquid biopsy is an excellent alternative for the detection of EGFR T790M mutation. However, this relatively novel method requires standardization and vigorous quality insurance. Thus, a standard set of guideline recommendations for liquid biopsy for EGFR mutation testing suitable for the Korean medical community is necessary. In this article, we propose a set of provisional guideline recommendations that was discussed and approved by the Cardiopulmonary Pathology Study Group of the Korean Society of Pathologists.

Metaplastic Meningioma Overspreading the Cerebral Convexity

Meningioma is relatively common, benign, and extra-axial tumor accounting for about 20% of primary brain and spinal cord tumors. The World Health Organization (WHO) classified these tumors into Grade I (benign), Grade II (atypical), and Grade III (anaplastic) meningioma. Grade I meningioma which is slowly growing tumor and have some rare subtypes. Among them, metaplastic subtype is defined as a tumor containing focal or widespread mesenchymal components including osseous, cartilaginous, lipomatous, myxoid or xanthomatous tissue, singly or in combinations. We report a rare metaplastic meningioma overspreading nearly whole cerebral convexity from main extra-axial tumor bulk in the parietal lobe.

Pulmonary Leukocytoclastic Vasculitis as an Initial Presentation of Myelodysplastic Syndrome / 결핵및호흡기질환

Systemic vasculitis involving the lung is a rare manifestation of myelodysplastic syndrome (MDS), and secondary vasculitis is considered to have poor prognosis. A 44-year-old man presented with fever and dyspnea of 1 month duration. A chest radiograph revealed bilateral multiple wedge shaped consolidations. In addition, the results of a percutaneous needle biopsy for non-resolving pneumonia were compatible with pulmonary vasculitis. Bone marrow biopsy was performed due to the persistence of unexplained anemia and the patient was diagnosed with MDS. We reported a case of secondary vasculitis presenting as non-resolving pneumonia, later diagnosed as paraneoplastic syndrome of undiagnosed MDS. The cytopenia and vasculitis improved after a short course of glucocorticoid treatment, and there was no recurrence despite the progression of underlying MDS.

Analysis of Mutations in Epidermal Growth Factor Receptor Gene in Korean Patients with Non-small Cell Lung Cancer: Summary of a Nationwide Survey

BACKGROUND: Analysis of mutations in the epidermal growth factor receptor gene (EGFR) is important for predicting response to EGFR tyrosine kinase inhibitors. The overall rate of EGFR mutations in Korean patients is variable. To obtain comprehensive data on the status of EGFR mutations in Korean patients with lung cancer, the Cardiopulmonary Pathology Study Group of the Korean Society of Pathologists initiated a nationwide survey. METHODS: We obtained 1,753 reports on EGFR mutations in patients with lung cancer from 15 hospitals between January and December 2009. We compared EGFR mutations with patient age, sex, history of smoking, histologic diagnosis, specimen type, procurement site, tumor cell dissection, and laboratory status. RESULTS: The overall EGFR mutation rate was 34.3% in patients with non-small cell lung cancer (NSCLC) and 43.3% in patients with adenocarcinoma. EGFR mutation rate was significantly higher in women, never smokers, patients with adenocarcinoma, and patients who had undergone excisional biopsy. EGFR mutation rates did not differ with respect to patient age or procurement site among patients with NSCLC. CONCLUSIONS: EGFR mutation rates and statuses were similar to those in published data from other East Asian countries.

Ruptured Cystic Artery Pseudoaneurysm as a Rare Cause of Massive Hemobilia / 대한췌담도학회지

Gastrointestinal bleeding from the biliary tree, called hemobilia, is an uncommon event. It may clinically present as hematemesis or melena. Ruptured cystic artery pseudoaneurysm is a rare cause of hemobilia, with 2 cases reported in Korea. We present this unusual condition in a 65-year-old man whose chief complaint was abdominal pain. His final diagnosis was ruptured cystic artery pseudoaneurysm, and he was successfully treated by transcatheter arterial embolization and laparoscopic cholecystectomy.

Innervation in women with uterine myoma and adenomyosis

OBJECTIVE: To determine if neurofilament (NF) is expressed in the endometrium and the lesions of myomas and adenomyosis, and to determine their correlation. METHODS: Histologic sections were prepared from hysterectomies performed on women with adenomyosis (n=21), uterine myoma (n=31), and carcinoma in situ of the uterine cervix. Full-thickness uterine paraffin blocks, which included the endometrium and myometrium histologic sections, were stained immunohistochemically using the antibodies for monoclonal mouse antihuman NF protein. RESULTS: NF-positive cells were found in the endometrium and myometrium in 11 women with myoma and in 7 with adenomyosis, but not in patients with carcinoma in situ of uterine cervix, although the difference was statistically not significant. There was no significant difference between the existence of NF-positive cells and menstrual pain or phases. The NF-positive nerve fibers were in direct contact with the lesions in nine cases (29.0%) of myoma and in five cases (23.8%) of adenomyosis. It was analyzed if there was a statistical significance between the existence of NF positive cells in the endometrium and the expression of NF-positive cells in the uterine myoma/adenomyosis lesions. When NF-positive cell were detected in the myoma lesions, the incidence of NF-positive nerve cells in the eutopic endometrium was significantly high. When NF-positive cell were detected in the basal layer, the incidence of NF-positive nerve cells in the myoma lesions and adenomyosis lesions was significantly high. CONCLUSION: We assume that NF-positive cells in the endometrium and the myoma and adenomyosis lesions might play a role in pathogenesis. Therefore, more studies may be needed on the mechanisms of nerve fiber growth in estrogen-dependent diseases.

Pericardial Amyloidosis Associated with Light-chain Myeloma / 대한내과학회지

Amyloidosis is characterized by the deposition of amyloid fibrils, which cause both functional and structural damage to organs. Cardiac involvement with amyloids is a common cause of secondary cardiomyopathy and leads to congestive heart failure. Pericardial amyloidosis usually occurs at the late or terminal stage of the disease after myocardial involvement, but it is extremely rare for pericardial amyloidosis to proceed with myocardial involvement. Here, we report a rare case of a 66-year-old patient with light-chain myeloma, who presented with pericardial effusion but no evidence of cardiomyopathy.

A Case of Pulmonary Artery Intimal Sarcoma Masquerading as Pulmonary Embolism / 결핵및호흡기질환

Pulmonary artery intimal sarcoma is a rare tumor with no characteristic symptoms. It is frequently misdiagnosed as pulmonary embolism. We report a case of pulmonary artery intimal sarcoma in a 48-year-old man with dyspnea, cough and blood-tinged sputum. He was initially suspected and treated as a pulmonary embolism. Computed tomography of the chest showed filling defects occupying the entire luminal diameter of the right and left pulmonary artery as well as extraluminal extension of the intraluminal mass. Surgical resection of the tumor confirmed pulmonary artery intimal sarcoma. After surgery, he received 8 cycles of combined chemotherapy consisting of doxorubicin and ifosfamide. After 8 cycles, Computed tomography of the chest showed interval regression of the residual tumor. Radiotherapy was done as total 6,000 cGy for 5 weeks, following the 8th chemotherapy. The patient's condition was successfully stabilized with chemotherapy and radiotherapy.

Duodenal Somatostatinoma: A Case Report and Review

Somatostatinomas are rare functioning carcinoid tumors that usually arise in the pancreas and duodenum. They are seldom associated with typical clinical symptoms; their diagnosis is confirmed only by histological and immunohistochemical studies and the presence of specific hormones. Two distinct clinicopathological forms of somatostatinoma exist: duodenal and pancreatic somatostatinomas. Clinically, compared to pancreatic somatostatinomas, duodenal somatostatinomas are more often associated with nonspecific symptoms and neurofibromatosis, but less often with somatostatinoma syndrome or metastasis. Histologically, duodenal somatostatinomas frequently have psammoma bodies in the tumor cells. We report a case of duodenal somatostatinoma in 58-year-old man with vague epigastric pain and nausea. He did not have diabetes, steatorrhea, or cholelithiasis. Abdominal computed tomography showed a 25-mm mass in the duodenum and 25-mm nodule in the liver. Endoscopic retrograde cholangiopancreatography showed a duodenal submucosal tumor. Although the endoscopic biopsies were free of malignancy, the patient subsequently underwent Whipple's operation for the duodenal mass. Examination revealed as a somatostatinoma using a special stain for somatostatin.

Testicular Epidermoid Cyst on Diffusion-Weighted MR Imaging and ADC Map : A Case Report

Testicular epidermoid cyst is a rare benign tumor, accounting for 1-2% of all testicular tumors. It can be cured by organ preserving surgery, so accurate preoperative diagnosis is very important for preventing unneccessary and extensive orchiectomy. We experienced a case of an 18-year-old man who presented with a painless lump in his right testis. The testicular mass showed an onion ring sign on ultrasonography. Computed tomography images showed the mass as a low attenuating lesion with curvilinear calcification. On Magnetic resonance imaging (MRI), the mass appeared as high signal intensity with internal alternating low signal intensity patterns on T2-weighted images. The mass was displayed as having homogeneous high signal intensity on diffusion magnetic resonance imaging and showed lower apparent diffusion coefficient values than normal testis parenchyma, similar to intracranial epidermoid cysts. Testicular MRI with DWI and ADC map can help to more accurately diagnose testicular epidermoid cyst.

Tricuspid Valvular Myxoma: Unusual Case of Tricuspid Valve Myxoma Mimicking Thrombus after Pulmonary Artery Embolectomy and Tricuspid Annuloplasty in Pulmonary Thromboembolism Patient

A 55-year-old man with massive pulmonary thromboembolism underwent thrombolysis, pulmonary artery embolectomy and tricuspid annuloplasty. Nine months later, a mobile echogenic intra-cardiac mass was found in the tricuspid valve. Because the patient had undergone annuloplasty, thrombosis was suspected as the most likely diagnosis and thrombolytic therapy was instituted. However, the size of the cardiac mass did not change and after surgical excision the mass was found to be a myxoma. Cardiac valvular tumors are uncommon and when they occur they are usually slow growing fibroelastomas. In this case, the rapid growing cardiac myxoma on the tricuspid valve was found after the occurrence of pulmonary thromboembolism. To our knowledge, this is first reported case of tricuspid valve myxoma in Korea.

Splenic infarction in a patient with autoimmune hemolytic anemia and protein C deficiency

Splenic infarction is most commonly caused by cardiovascular thromboembolism; however, splenic infarction can also occur in hematologic diseases, including sickle cell disease, hereditary spherocytosis, chronic myeloproliferative disease, leukemia, and lymphoma. Although 10% of splenic infarction is caused by hematologic diseases, it seldom accompanies autoimmune hemolytic anemia (AIHA). We report a case of a 47-year-old woman with iron deficiency anemia who presented with pain in the left upper abdominal quadrant, and was diagnosed with AIHA and splenic infarction. Protein C activity and antigen decreased to 44.0% (60-140%) and 42.0% (65-140%), respectively. Laboratory testing confirmed no clinical cause for protein C deficiency, such as disseminated intravascular coagulation, sepsis, hepatic dysfunction, or acute respiratory distress syndrome. Protein C deficiency with splenic infarction has been reported in patients with viral infection, hereditary spherocytosis, and leukemia. This is a rare case of splenic infarction and transient protein C deficiency in a patient with AIHA.

Xanthogranulomatous Pancreatitis Presents as a Solid Tumor Mass: A Case Report

Xanthogranulomatous inflammation (XGI) is a rare, idiopathic process in which lipid-laden histiocytes are deposited at various locations in the body. Although XGI has been reported to occur in various organs such as the gallbladder, kidney, bone, stomach, colon, appendix, lymph nodes, urachus, and urinary bladder and in soft tissues, xanthogranulomatous pancreatitis (XGP) is extremely rare. Herein, we report a case of XGP occurring in a 70-yr-old woman, who presented with abdominal pain for several months. On physical examination, mild epigastric tenderness was noted. Abdomen CT scan revealed a low attenuated mass in uncinate process of pancreas, suggesting malignant lesion. Whipple's operation was performed and the final pathologic diagnosis was XGP. The patient's post-operative course was uneventful, and no recurrence was found within 7 months of the operation. When a pancreatic mass does not show clinico-radiological features typical of common pancreatic neoplasms, XGP should be considered for a differential diagnosis.

Diagnostic Utility of AMACR and Claudin-7 for the Classification of Renal Cell Carcinoma

BACKGROUND: The histologic classification of renal cell carcinoma (RCC) is based on the cytoarchitectural features, yet sometimes this requires correlation with the immunophenotype. Alpha-methylacyl-CoA racemase (AMACR) and claudin-7 have recently been introduced as useful markers that are frequently expressed in papillary RCC (PRCC) and chromophobe RCC (ChRCC), respectively. The aims of this study are to evaluate the expressions of AMACR and claudin-7 in RCCs and to investigate whether they are helpful for making the histological classification of RCCs. METHODS: Immunohistochemistry for CD10, RCC marker, cytokeratin (CK)7, CD117, AMACR and claudin-7 was performed for 104 RCCs, and these consisted of 54 clear cell RCCs (CCRCC), 26 PRCCs and 24 ChRCCs. RESULTS: For diagnosing PRCC, the sensitivity and specificity of AMACR were 92.3% and 71.8%, respectively, and using AMACR(+)/CK7(+), the specificity was increased by 23.1% to 94.9%. For diagnosing ChRCC, the sensitivity and specificity of claudin-7 were 91.7% and 78.8%, respectively, and using claudin-7(+)/AMACR(-), the specificity was significantly improved (to 96.3%). For diagnosing CCRCC, CK7(-)/claudin-7(-)/CD117(-) was the most useful immunohistochemical panel (sensitivity, 96.3%; specificity, 98%). CONCLUSIONS: AMACR and claudin-7 are helpful markers for the histologic classification of RCCs, and their diagnostic utility is strengthened when they are used as an immunohistochemical panel, AMACR(+)/CK7(+) for PRCC, claudin-7(+)/AMACR(-) for ChRCC and CK7(-)/claudin-7(-)/CD117(-) for CCRCC.

Gnathostoma hispidum Infection in a Korean Man Returning from China

Human Gnathostoma hispidum infection is extremely rare in the world literature and has never been reported in the Republic of Korea. A 74-year-old Korean man who returned from China complained of an erythematous papule on his back and admitted to our hospital. Surgical extraction of the lesion and histopathological examination revealed sections of a nematode larva in the deep dermis. The sectioned larva had 1 nucleus in each intestinal cell and was identified as G. hispidum. The patient recalled having eaten freshwater fish when he lived in China. We designated our patient as an imported G. hispidum case from China.

A Case of Pancreatitis Presenting with Pancreatic Panniculitis: A Case Report

Pancreatic panniculitis is a rare disease in which necrosis of fat in the panniculus and other distant foci occur in the setting of pancreatic disease. The most common pancreatic disorders associated with pancreatic panniculitis are acute and chronic pancreatitis, especially the alcohol-related types and pancreatic carcinoma. We now report a case of pancreatic panniculitis in which skin eruptions, not abdominal symptoms, were the presenting symptom. A 45-year-old man presented with multiple erythematous tender subcutaneous nodules on the shins, arthralgia of both ankles and swelling of the left foot. He was diagnosed as having pancreatitis 15 months ago. Histopathologic findings of a skin biopsy showed focal fat necrosis with anucleated "ghost-like" fat cells with a thick shadowy wall. Laboratory evaluation revealed an increase in serum amylase and lipase. We diagnosed this patient's problem as pancreatic panniculitis associated with a relapse of pancreatitis.

Giant Cell Tumor-like Proliferation Associated with Renal Staghorn Calculi: A Case Report

A 62-year-old man with left flank pain and hematuria was shown to have a staghorn stone in left renal pelvis. Grossly, renal pelvis and calyces were markedly dilated with cystic and hemorrhagic degeneration and renal parenchyma was atrophied. A tumor-like mass was located in a hemorrhagic cyst of the renal upper pole. This mass consisted of giant cells and stromal cells mimicking a giant cell tumor of bone. This giant cell tumor-like proliferation may represent a response to hemorrhage into a cystic cavity. Recognition of this finding is important to avoid the over-diagnosis of neoplastic lesions.