Parosteal Lipoma Associated with a Growing Osteochondroma of the Right Ilium

Parosteal lipoma is a rare type of lipoma, the incidence being approximately 0.3% of all lipomas. Moreover, parosteal lipoma coexisting with osteochondroma is extremely rare. A few cases with coexistence of osteochondroma and parosteal lipoma have been reported and they were thought to be reactive changes of adjacent bone by parosteal lipoma. However, temporal relationship of these tumors could not be explained. Here, we report a case of parosteal lipoma associated with osteochondroma of the right ilium developed over 6 years, with follow-up radiographs.

Parosteal Lipoma Associated with a Growing Osteochondroma of the Right Ilium

Parosteal lipoma is a rare type of lipoma, the incidence being approximately 0.3% of all lipomas. Moreover, parosteal lipoma coexisting with osteochondroma is extremely rare. A few cases with coexistence of osteochondroma and parosteal lipoma have been reported and they were thought to be reactive changes of adjacent bone by parosteal lipoma. However, temporal relationship of these tumors could not be explained. Here, we report a case of parosteal lipoma associated with osteochondroma of the right ilium developed over 6 years, with follow-up radiographs.

An Intraosseous Schwannoma Combined with a Subchondral Fracture of the Femoral Head: a Case Report and Literature Review

Schwannomas are benign nerve sheath tumors that are typically located in soft tissue. Occasionally, schwannomas involve osseous structures. These intraosseous schwannomas are generally benign neoplasms that account for less than 0.2% of primary bone tumors. Schwannomas are very rarely observed in long bones. We present a case of a schwannoma affecting the proximal femur with a coincident subchondral fracture of the femoral head. A 38-year-old-male presented with left hip pain without deteriorating locomotor function. Plain film radiographs displayed a lobulating contoured lesion within the intertrochanteric portion of the femur. The magnetic resonance imaging (MRI) scans showed a tumor occupying the intertrochanteric region. Diffuse bone marrow edema, especially in the subchondral and head portions of the femur that was possibly due to the subchondral insufficiency fracture was also noted. The lesion was surgically excised and bone grafting was performed. Histologically, there was diffuse infiltrative growth of the elongated, wavy, and tapered cells with collagen fibers, which are findings that are characteristic of intraosseous schwannoma. Although very rare, intraosseous schwannoma should be included in the differential diagnosis of radiographically benign-appearing, non-aggressive lesions arising in the femur. The concomitant subchondral fracture of the femoral head confounded the correct diagnosis of intraosseous schwannoma in this case.

Amyloid Arthropathy of the Hip Joint Associated with Multiple Myeloma: A Case Report / 대한고관절학회지

Amyloidosis is a disease characterized by the deposition of non-soluble fibrous protein in multiple tissues with a number of possible causes. This protein deposition can occur in any tissue, yet is most commonly seen in kidneys, heart, and gastrointestinal tracts. However, invasion to bone tissues is not often reported. The deposition of amyloid proteins in bone tissues may result in joint pain and pathological fractures; it is important to elucidate the causes and detect early to determine prognosis and treat optimally. In the present case report, with relevant literature review, the authors report a case of total hip arthroplasty in an amyloidosis patient.

A Case of Primary Gastric Amyloidosis with Fulminant Heart Failure / 대한소화기학회지

A 53-year-old woman was admitted with epigastric discomfort and weakness. Laboratory examination at admission showed mild anemia and proteinuria. Esophagogastroduodenoscopy revealed marked mucosal atrophy, diffuse nodularity and granular appearance with mucosal friability. Biopsy was performed on the antrum and body of the stomach. On the next day, the patient began to complain of severe dyspnea, and hypoxia was present on pulse oximetry. Therefore, emergency echocardiography was conducted and it showed restrictive cardiomyopathy along with thrombus in the left atrium. With time, heart failure was aggravated despite intensive management. The result of gastric biopsy revealed amyloid deposits which stained positively with Congo red. On immunohistochemistry study, kappa and lambda chain were present. In addition, kappa chain was significantly elevated in urine and serum on electrophoresis. Although the patient was finally diagnosed as having primary gastric amyloidosis with restrictive cardiomyopathy, her general condition rapidly deteriorated and died at 12th hospital day. When obscure gastric lesion is encountered, performing gastric biopsy is strongly recommended since it be primary gastric amyloidosis. Herein, we present an unusual case of primary gastric amyloidosis.

IDH Mutation Analysis in Ewing Sarcoma Family Tumors

BACKGROUND: Isocitrate dehydrogenase (IDH) catalyzes the oxidative decarboxylation of isocitrate to yield alpha-ketoglutarate (alpha-KG) with production of reduced nicotinamide adenine dinucleotide (NADH). Dysfunctional IDH leads to reduced production of alpha-KG and NADH and increased production of 2-hydroxyglutarate, an oncometabolite. This results in increased oxidative damage and stabilization of hypoxia-inducible factor alpha, causing cells to be prone to tumorigenesis. METHODS: This study investigated IDH mutations in 61 Ewing sarcoma family tumors (ESFTs), using a pentose nucleic acid clamping method and direct sequencing. RESULTS: We identified four cases of ESFTs harboring IDH mutations. The number of IDH1 and IDH2 mutations was equal and the subtype of IDH mutations was variable. Clinicopathologic analysis according to IDH mutation status did not reveal significant results. CONCLUSIONS: This study is the first to report IDH mutations in ESFTs. The results indicate that ESFTs can harbor IDH mutations in previously known hot-spot regions, although their incidence is rare. Further validation with a larger case-based study would establish more reliable and significant data on prevalence rate and the biological significance of IDH mutations in ESFTs.

Multifocal Osteosarcoma of the Skull: Multiple Primary or Metastatic? A Case Report

Osteosarcoma of the skull is a very rare condition. Moreover, it is extremely rare for osteosarcoma to present as multiple lesions confined to the skull. A 58-year-old woman was admitted with two masses in the parietal area of the skull, accompanied by mild headache and tenderness. Imaging revealed two masses with a heterogeneous consistency in the cranial bones. Excision craniectomy was performed and the pathology was consistent with osteoblastic osteosarcoma. Two nodules in the heart were found on routine follow-up imaging while the patient was undergoing chemotherapy. The nodules were biopsied and found to be metastatic osteosarcoma.

Recurrent Thyroid Papillary Carcinoma in Children Under Ten Years Old: Report of Two Cases and Literature Review

Papillary thyroid carcinoma (PTC) in children under ten years old is very rare. To date, 18 cases of PTC in children under ten years old (including our two cases) have been reported in Korea. Here, we describe two cases of recurrent PTC with follicular variant and conventional type in an 8-year-old boy and a 7-year-old boy, respectively, and discuss clinicopathologic and molecular characteristics that differ in pediatric patients from adults.

Actinomycotic Brain Abscess Developed 10 Years after Head Trauma

No abstract available.

Spinal Cord Injury due to the Giant Cell Tumor of the Second Thoracic Vertebra: A Case Report

Giant cell tumor (GCT) is a relatively rare neoplasm. In GCT, the bone affection of the axial skeleton is extremely rare. Most GCT arises in the meta-epiphyseal ends of the long bones. Its peak incidence is between 30 to 40 years of age. GCT is usually classified as benign, but shows locally aggressive behavior and may occasionally undergo a malignant transformation. The patients with GCT in the spine often complain of the lower back pains, as the tumors primarily involve the sacrum. We report a case of an adolescent female complaining of the upper back pain with a sudden weakness of the lower extremities, later diagnosed with the GCT of the T2 vertebra. The present patient showed American Spinal Injury Association Impairment Scale (AIS) D before the surgery, which changed to AIS E after the treatments including the surgery, radiation therapy and rehabilitation.

Ultrasonographic finding Paratesticular Embryonal Rhabdomyosarcoma: Case Reports

Rhabdomyosarcoma (RMS) may arise from anywhere in the body, and RMS has been recognized as one of the most frequent forms of childhood solid tumors, occurring with a frequency similar to that of Wilms' tumors and neuroblastomas. However, RMS arising in the paratesticular region is rare so that an ultrasound appearance of paratesticular RMS has rarely been reported. We report the US findings of paratesticular embryonal RMS in 15 and 19 year old males.

Multifocal Renal Cell Carcinoma of Different Histological Subtypes in Autosomal Dominant Polycystic Kidney Disease

Renal cell carcinoma (RCC) in autosomal dominant polycystic kidney (ADPKD) is rare. To date, 54 cases of RCC in ADPKD have been reported. Among these, only 2 cases have different histologic types of RCC. Here we describe a 45-year-old man who received radical nephrectomy for multifocal RCC with synchronous papillary and clear cell histology in ADPKD and chronic renal failure under regular hemodialysis. The case reported herein is another example of the rare pathological finding of RCC arising in a patient with ADPKD.

Differential Expression of CXCR4 in Conventional High-grade and Low-grade Central Osteosarcoma and Its Prognostic Implications / 대한골관절종양학회지

PURPOSE: The chemokine receptor CXCR4 has been reported to be aberrantly expressed in human cancer and has been shown to participate in cancer metastasis. We compared the expression of CXCR4 in conventional high-grade and low-grade central osteosarcomas, and determined if an association between CXCR4 expression and prognosis could be made. MATERIALS AND METHODS: We performed the immunohistochemistry for CXCR4 in a total of 63 patients with osteosarcoma and determined the relationships according to the clinicopathologic variables and overall survival rates. RESULTS: CXCR4 was detected in 76.3% of conventional high-grade osteosarcoma patients and in 36% of low-grade central osteosarcomas. Diffuse expression was noted in 47.4% of the high-grade osteosarcomas and all low-grade cases were focal positive. CXCR4 expression was significantly correlated with histologic grade (p<0.0001). While overall survival rate was reduced significantly with increased CXCR4 expression (p=0.0058), higher histologic grade (p<0.0001), and younger age (p=0.0140), survival rate did not correlate with gender, tumor size, or AJCC stage. CONCLUSION: Our results suggest that CXCR4 expression is associated with higher-grade tumors and with poor prognosis for osteosarcoma patients.

Novel Mutation in PRKAR1A in Carney Complex

A case of Carney complex in a Korean patient is presented. The patient had the characteristics of Carney complex including skin lesions, positive family history, and multiple myxomas including a superficial angiomyxoma in the perianal area. An extensive genetic analysis revealed a novel mutation in the protein kinase A type I-a regulatory subunit (PRKAR1A) gene, but not in the phosphodiesterase type 11A (PDE11A) gene. This is the first case wherein extensive genetic studies were performed in a patient with Carney complex in Korea.

Chondroblastoma of the Lumbar Spine: A Case Report and Review of the Literature

We report a case of chondroblastoma arising in the lumbar spine in a 25-year-old man who presented with low back pain of 5 years duration. Plain radiography and computed tomography revealed a well-defined osteolytic mass surrounded by marginal sclerosis in the third lumbar vertebra. The mass encroached on the left neural foramen on magnetic resonance imaging. Histologically, the tumor consisted of round to oval cells with eosinophilic cytoplasm and randomly scattered osteoclastic type giant cells. There were characteristic chicken-wire calcification and aneurysmal bone cyst-like changes. Chondroblastomas of the lumbar spine are extremely rare, and only nine cases have been reported. Spinal chondroblastoma should be distinguished from other benign bone tumors, because it tends to show aggressive biological behavior with high recurrence and mortality rates.

The Analysis of Co-authorship and Networks among the Korean Pathologists

BACKGROUND: To evaluate the characteristics of the co-authorship and its network within the Korean Pathologists' Society. METHODS: In the KoreaMed database, 11,420 articles and 72,478 authors from 1991 to 2010 were searched. The patterns of co-authorship of the authors and institutions were analyzed to build a network matrix. The network centrality indices were measured with UCINET 6.0 and sociogram, and were drawn with Netdraw 5.0. KeyPlayer 1.44 was used for key player analysis. RESULTS: The number of articles that pathologist participated in increased; however, the number of articles that the pathologists are the first author did not increase. The centrality degrees from 1991 to 2010 were 4.16% and 0.3% for the institutions and authors network, respectively. From 1991 to 2000, Seoul National University had the highest degree of centrality and was a key player. However, from 2001 to 2010, Ulsan replaced the position. For the authors, Chi, Je Geun was highest centrality author and key player during the 1991 to 2000 time period. From 2001 to 2010, Yoo, Jinyoung had the highest degree of centrality and Kim, Na Rae was a key player. Overall, most of the centrality indices were occupied by only a few institutions and authors. CONCLUSIONS: The network among the pathologist society is a typical small world society.

The Global Histone Modification Patterns of Osteosarcoma

BACKGROUND: Epigenetic alteration may affect a patient's prognosis by altering the development and progression of the tumor. Some recent reports have identified a correlation between histone modification and patient outcome. However, no studies have been conducted on global histone modification in osteosarcomas. METHODS: We investigated histone modification in 54 cases of osteosarcoma by performing immunohistochemical staining. The immunohistochemical expression of four histone modification markers, acetylated H4 lysine 12 (H4K12Ac), acetylated H3 lysine 18, trimethylated H3 lysine 27, and dimethylated H3 lysine 4 were evaluated. RESULTS: High H4K12Ac expression was correlated with patient age (p=0.011). However, the other histone modification markers showed no correlation with any of the clinicopathological data such as survival, tumor grade, tumor site, metastasis, age, or gender. CONCLUSIONS: Our study showed that all four histone modification markers are expressed in osteosarcoma (median expression rate, 40 to 60%). However, we did not find a correlation with the clinicopathological factors except for age. Further study to evaluate the reason for the association between H4K12Ac and patient age is needed.

Type and Incidence of Soft Tissue Sarcomas in Korea: 2001-2007

BACKGROUND: The Korean Bone and Soft Tissue Pathology Study Group of the Korean Society of Pathologists conducted a nationwide retrospective analysis of soft tissue sarcoma (STS) to provide the clinicopathologic characteristics of STS within the population of the Republic of Korea. METHODS: The cases of STS were collected during a 7-year period (2001-2007) from 19 institutes in Korea. All cases were classified according to the histologic criteria proposed by the World Health Organization. Clinicopathologic data were reviewed. RESULTS: Data from 722 patients (median age, 50 years) were collected. Data showed a slight male predominance. The most frequent types of STS in decreasing order were liposarcoma, malignant fibrous histiocytoma, leiomyosarcoma, and synovial sarcoma. STS occurred throughout the body, although approximately half (47.8%) were located in the extremities. The majority of STS was histologically classified as high grade with a large tumor size (>5 cm). The overall survival rate for the patients was 76.3% (median follow-up time, 26 months; range, 1 to 89 months). Histologic grade, tumor size, American Joint Committee on Cancer stage, tumor site, and resection status were prognostic. Significant independent adverse prognostic factors were large tumor size (>5 cm) and tumor site other than extremities. CONCLUSIONS: We reported the distribution and characteristics of STS in the Republic of Korea.

14-bp Insertion/Deletion Polymorphism of the HLA-G Gene in Osteosarcoma Patients

BACKGROUND: The major histocompatibility complex class I, G (human leukocyte antigen-G [HLA-G]) gene plays a vital role in the suppression of immune responses. Recently, a number of studies have reported an association between HLA-G and diseases (pregnancy complications, organ transplantation, and tumors). Some of the studies have revealed that the 14-bp insertion/deletion polymorphism might be associated with various diseases. The aim of the present study was to explore a possible influence of the 14-bp insertion/deletion polymorphism on osteosarcoma. METHODS: Genomic DNA was extracted from 75 formalin-fixed, paraffin-embedded tumor tissues derived from patients with conventional osteosarcoma (OSA) and 183 peripheral blood samples of healthy controls. Fifty-eight cases were South Korean patients with OSA and 17 cases were Argentine patients with OSA. The HLA-G 14-bp insertion/deletion polymorphism at exon 8 of the HLA-G locus was analyzed by polymerase chain reaction. RESULTS: There was a significantly different distribution profile for the 14-bp genotypes between the Korean OSA and Korean control groups. Specifically, there were more heterozygote 210 bp/224 bp genotypes in the Korean OSA group when compared to the Korean control group (62.1% vs 40.4%, p=0.002). CONCLUSIONS: The results suggest that HLA-G heterozygote patients may be more susceptible to OSA in the Korean population.

A Rare Case of Epiphyseal Chondromyxoid Fibroma of the Proximal Tibia

Chondromyxoid fibroma is an uncommon benign cartilaginous tumor of the bone. It occurs most frequently in the metaphysis of long tubular bones, and an epiphyseal location is exceedingly rare. We present here an unusual case of a chondromyxoid fibroma that occurred in the epiphysis of the proximal tibia with an open growth plate. MR imaging findings of this tumor, which has, to the best of our knowledge, never been described in an epiphyseal location, makes the present case unique.