Juxtacortical chondromyxoid fibroma in the small bones: two cases with unusual location and a literature review

Chondromyxoid fibroma is a rare bone tumor of cartilaginous origin, representing less than 1% of all bone tumors. It preferentially arises in the eccentric location of the metaphysis of a long tubular bone. Juxtacortical locations are reported infrequently in the long bones and even more rarely in short tubular bones, with only three cases documented. Here we present two new cases of juxtacortical chondromyxoid fibroma in the small bones. One was an intracortical osteolytic lesion of the metatarsal bone of the foot with degenerative atypia that histologically should be differentiated from chondrosarcoma. The other was a phalangeal mass protruding into the interphalangeal joint of the hand, which had been labeled mistakenly as a soft tissue mass preoperatively. These cases illustrated that chondromyxoid fibromas have various the manifestations and should be included in the differential diagnosis of an osteolytic lesion or an exophytic mass in the small bones.

Fibrocartilaginous mesenchymoma with an unusual location in the rib

Fibrocartilaginous mesenchymoma is a rare bone tumor, with fewer than 35 cases reported in the literature since 1984. This tumor usually occurs in the long bones of children and adolescents. In the current case, the tumor affected a rib. A 17-year-old boy presented with a mass in the right fifth rib. Radiologic findings revealed an osteolytic mass with cortical destruction and calcification; en bloc resection was performed. The tumor showed three distinct histologic features: bland spindle cell proliferation, benign cartilage nodules, and epiphyseal plate-like enchondral ossification. The pathologic diagnosis was fibrocartilaginous mesenchymoma. The patient remains free of disease 1 year after the surgery. Pathological diagnosis of fibrocartilaginous mesenchymoma can be challenging, especially when the tumor occurs in an unusual site. When any fibro-osseous lesion with a cartilaginous component is encountered, the possibility of fibrocartilaginous mesenchymoma should be considered because of its locally aggressive behavior.

Rosette-forming epithelioid osteosarcoma in the rib: a rare case of location and morphology

The rib is an unusual location for osteosarcoma and is reported in only 2% of all cases. The major histological variants of osteosarcoma are osteoblastic, chondroblastic, and fibroblastic, with a few rare variants including one epithelioid type. This report describes a 44-year-old male with an osteolytic mass in the right seventh rib. Histological examination revealed osteosarcoma with unique features of epithelioid appearance and rosette structures. To the best of our knowledge, this is the first reported case of a rosette-forming osteosarcoma of the rib that showed epithelioid morphology. Despite successful surgery, the patient’s prognosis was poor because this malignancy had an unusual location within the axial skeleton and was a rare histological variant.

Primary Cutaneous Mucinous Carcinoma with Extramammary Paget’s Disease: Eccrine or Apocrine?

Primary cutaneous mucinous carcinoma (PCMC) is an uncommon tumor of the sweat gland origin. The occurrence of PCMC is mostly in middle-aged and older patients, with a slight male predominance. Most cases of PCMC arise on the head, with a preference for eyelids. The histogenesis of PCMC, whether eccrine or apocrine, remains controversial. We report a rare case of PCMC with secondary extramammary Paget’s disease in the groin of a 75-year-old man, which favored an apocrine origin. Furthermore, based on a review of the literature, we provide several histologic clues that can be used to differentiate PCMC from metastatic mucinous carcinoma.

Synovial Sarcoma of the Extremities / 대한정형외과학회잡지

PURPOSE: Synovial sarcoma is an uncommon malignant soft tissue tumor mostly prevalent in young adults. Previous studies analyzing the prognostic factors have been limited due to the inclusion of heterogenous cohorts of patients with nonextremity and recurrent tumors. The purpose of this study was to determine the independent prognostic factors for the treatment methods associated with local recurrence and metastasis of primary synovial sarcoma localized to the extremities. MATERIALS AND METHODS: Between April 1999 and June 2014, a total of 79 patients were diagnosed with synovial sarcoma, of which 73 underwent wide excision at Kosin University Gospel Hospital and were followed-up for 60 months (24-72 months). The analyzed prognostic factors were treatment methods for local recurrence and metastasis during the postoperative follow-up period. The Cox regression model was used for multivariate analysis. RESULTS: For local recurrence according to the treatment methods, 9 recurrences occurred in 9 surgical treatments, 8 in 20 chemotherapy added surgical treatments, 13 in 20 radiotherapy added surgical treatments, and 4 in 24 chemotherapy with radiotherapy added surgical treatments. For metastasis according to the treatment methods, 9, 5, 12, and 2 metastases occurred respectively. Chemotherapy had statistical significance in multivariate analysis (p<0.0001). CONCLUSION: Adjuvant chemotherapy can be useful for the treatment of synovial sarcoma.

The Clinical Characteristics of Giant Cell Tumor of Bone with Aneurysmal Bone Cyst / 대한정형외과학회잡지

PURPOSE: The purpose of this study was to evaluate the difference in clinical features, process, and prognosis depending on the presence of secondary aneurysmal bone cysts (ABCs) in patients with giant cell tumors. MATERIALS AND METHODS: A total of 33 patients who underwent surgery for giant cell bone tumors between March 2009 and April 2013 were selected. Data on clinical features were obtained from medical records and pathological and radiological review, including age, sex, location, and size of the tumor, and Campanacci grade, as well as whether there was any pathological fracture, local recurrence, distant metastasis, or malignant transformation. The Student t-test and Fisher exact test were used for comparison of the differences in clinical features by the presence or absence of ABCs. RESULTS: Local recurrence occurred in 6 of the 33 cases, 3 each were in the groups with and without ABCs; however, the difference was not statistically significant. In total, nine cases had pathological fractures, seven were in the group with ABC and two were in the group without ABC (p=0.013). No statistically significant differences in age, sex, location and size of the tumor, or Campanacci grade were observed between the groups with and without ABCs. CONCLUSION: There was no difference in the frequency of local recurrence between the groups with and without ABCs. However, pathological fracture occurred more frequently in the group with ABCs compared to the group without ABCs.

Late Bone Metastasis of Histologically Bland Struma Ovarii: The Unpredictability of Its Biologic Behavior

No abstract available.

Expression of MUC1 and MUC4 in Gallbladder Adenocarcinoma

BACKGROUND: Recent reports have indicated that overexpression of mucin (MUC) 1 and/or MUC4 correlates with the occurrence and progression of extra-hepatobiliary malignancy. In this study, we investigated the expression of MUC1 and MUC4 and their prognostic significance in gallbladder adenocarcinoma. METHODS: We examined 54 surgical gallbladder adenocarcinoma samples by immunohistochemistry for MUC1 and MUC4 expression. Staining was evaluated as a sum score of extent and intensity, dividing the samples into low and high expression groups. RESULTS: The low expression group for both MUC1 and MUC4 was 10 samples (18.5%), and the high expression group was 44 samples (81.5%). High MUC1 expression was significantly correlated with more differentiated tumors (p=0.033), whereas high expression of MUC4 correlated with negative nodal status (p=0.012). Other pathological features were not correlated with MUC expression. Multivariate cox regression analysis showed that neither MUC1 nor MUC4 expression correlated with survival. CONCLUSIONS: Although there were some correlations found, a prognostic role for either MUC1 or MUC4 expression in gallbladder carcinoma was not identified in this study. Further investigation is required.

Clinicopathologic Implications of PIWIL2 Expression in Colorectal Cancer

BACKGROUND: There are no established reports about the expression of the Piwil gene, a subfamily of the Piwi gene involved in RNA silencing and self-renewal, in colorectal carcinomas. It is known that the degree of PIWIL2 expression is higher in colorectal carcinomas. But its clinicopathologic significance remains undetermined. This study reassessed the relationship between PIWIL2 expression and the clinicopathologic parameters in colorectal carcinomas. METHODS: An immunohistochemistry of PIWIL2 expression was done in 60 cases of colorectal carcinoma. This was followed by an analysis of the correlation between PIWIL2 expression and clinicopathologic features and a survival analysis. RESULTS: There were 44 cases (73.3%) where the degree of PIWIL2 expression was relatively higher. The high degree of PIWIL2 expression was significantly correlated with the lower degree of differentiation (p=0.039), deep invasion (p=0.019) and perineural invasion (p=0.027). The overall survival was longer in patients with the lower degree of PIWIL2 expression than in those with the higher degree of PIWIL2 expression. CONCLUSIONS: Our results showed that the degree of PIWIL2 expression was relatively higher in colorectal carcinomas and it was significantly correlated with variable clinicopathologic indicators for a poor prognosis. This indicates that PIWIL2-positive cells contribute to the progression of colorectal cancer.