Vascular soft-tissue sarcomas: a prognostic model from a retrospective single-center study.

BACKGROUND: Vascular soft-tissue sarcomas are a rare form of sarcoma. Malignant subtypes exhibit a variety of biological behaviors. We describe the clinicopathological characteristics and patient survival outcomes of malignant vascular soft-tissue sarcomas. METHODS: We conducted a retrospective study on a cohort of 84 patients diagnosed with vascular tumors by histological examination at the Yonsei University College of Medicine between April 1987 and August 2011. The primary end point was overall survival (OS). RESULTS: The angiosarcoma patients had a significantly shorter OS than the patients with other subtypes of sarcomas (59.0 and 142.7 months, respectively; p < 0.001). Upon multivariate analysis of survival in patients who underwent surgical resection, the following independent prognostic factors were identified: primary site (trunk, p = 0.001), age (older than 65 years, p < 0.001), pathology (angiosarcoma, p = 0.006) and R2 resection (p = 0.002). CONCLUSION: The independent prognostic factors for shorter survival are the trunk as the primary site, malignant angiosarcoma and age (>65 years). Complete excision should be attempted for providing a survival advantage in the patients with localized disease. In addition, bleeding episodes are much more frequent in patients with a poor survival outcome.

Prognostic implications of anaplastic lymphoma kinase gene aberrations in rhabdomyosarcoma; an immunohistochemical and fluorescence in situ hybridisation study.

BACKGROUND: We investigated the diagnostic and prognostic usefulness of anaplastic lymphoma kinase (ALK) expression in Asian rhabdomyosarcoma (RMS) patients. PATIENTS AND METHODS: A total of 38 RMS tissue samples were collected over a 14-year period (1998-2012). ALK protein expression and gene copy number were analysed by immunohistochemistry (IHC) and fluorescence in situ hybridisation (FISH). RESULTS: Ten of the 38 RMS patients (26.3%) showed positive ALK protein expression. ALK protein expression was predominantly positive in alveolar RMS (ARMS) compared with embryonal RMS (ERMS) (80% vs 20%, p=0.03). ALK protein expression was statistically associated with ARMS histology, metastatic disease at diagnosis, and primary trunk site. In FISH analysis, no translocations were detected and ALK gene copy number gain was observed more frequently in ARMS than in ERMS (40% vs 17%). The ALK-positive group showed inferior overall survival (OS) compared with ALK-negative group (p=0.014) for both alveolar and embryonal RMS patients. In multivariate analysis, positive ALK expression was an independent prognostic factor for OS (p=0.02; HR, 3.1; 95% CI 1.2 to 8.3). There was a significant strong positive correlation between ALK gene copy number and protein expression (Spearman's r<0.001, r=0.77). CONCLUSIONS: We demonstrated that ALK protein expression is statistically associated with ARMS histology, metastatic disease at diagnosis and primary trunk site. Additionally, ALK expression was an independent prognostic factor for worse survival. There was a strong correlation between IHC and FISH. Further studies are needed to evaluate the potential diagnostic and therapeutic role of ALK expression in RMS.

A case report of xanthogranulomatous osteomyelitis of the distal ulna mimicking a malignant neoplasm.

PATIENT: Male, 59 FINAL DIAGNOSIS: Xanthogranulomatous osteomyelitis Symptoms: Painful swelling in the wrist Medication: Drug history of antifulgal agents Clinical Procedure: Excisional biopsy Specialty: Radiology. OBJECTIVE: Rare disease. BACKGROUND: Xanthogranulomatous inflammation is a chronic inflammatory disease in which bone involvement is extremely rare. Bone involvement of xanthogranulomatous inflammation, termed xanthogranulomatous osteomyelitis (XO), often presents as a mass-like lesion extending to adjacent structures, which can mimic infiltrative carcinoma. CASE REPORT: We present a case of XO in the ulna, which mimicked a neoplasm. A 59-year-old man presented with a 2-month history of painful swelling in the right wrist. Plain radiography and CT showed an osteolytic lesion at the distal ulna. MRI revealed a soft-tissue mass with lobulated margins and contrast enhancement. Intense uptake in bone scan and PET suggested malignancy. An excisional biopsy from the representative area resulted in a pathology diagnosis of XO. CONCLUSIONS: Gross and radiologic manifestations of XO can mimic neoplasm. XO generally has benign prognosis, contrary to malignant bone tumor. Therefore, biopsy and histopathological confirmation are necessary for proper management.

Cytogenetics and prognosis for uveal melanoma in Korean patients.

PURPOSE: To determine the rate of mortality for uveal melanoma in the Korean population and assess whether it correlates with cytogenetic data, and clinical and histopathological factors. METHODS: A retrospective review of medical files from 33 uveal melanoma patients who underwent enucleation at Yonsei University, Seoul, Korea, between January, 1994 and December, 2009 was performed. Dual-colour fluorescence in situ hybridization was performed using centromeric probes for chromosome 3 and 8 in archived patient tissues. RESULTS: The mean age of patients was 53.2 years (range; 29-78), and the mean largest basal diameter was 11.8 mm (range; 6.0-18.1). Of the 20 tissue blocks with available cytogenetic data, there was monosomy 3 in seven (35%) and polysomy 8 in four (20%). The presence of monosomy 3 (p < 0.001), polysomy 8 (p < 0.001), ciliary body involvement (p < 0.001), a mitotic rate ≥5/40 high-power fields (p = 0.006), closed extravascular matrix loop (p = 0.025), large basal tumour diameter (p = 0.029) and epithelioid cellularity (p = 0.038) predicted melanoma-related mortality. The median time from enucleation to liver metastasis was 16 months (range; 10-70), and the median survival after metastasis was 5 months (range; 1-9). The Kaplan--Meier survival curve estimated the metastatic death rate to be 22% for 5 years and 30% for 10 years. CONCLUSIONS: Korean patients with uveal melanoma are younger and appear to exhibit no worse prognosis than Caucasian. Cytogenetic abnormality of chromosome 3 and 8 highly predicted metastatic death.

A choroidal schwannoma confirmed by surgical excision.

Schwannomas rarely present as intraocular tumors and are often misdiagnosed as malignant melanoma. We describe a choroidal schwannoma confirmed by sclerouvectomy. A 30-year-old woman presented with a large nonpigmented intraocular mass of the choroid in the right eye and underwent surgical excision by sclerouvectomy. Histologically, the tumor was composed of a mixture of cellular solid components (Antoni A) and loose myxoid components (Antoni B). The tumor was eventually diagnosed as a schwannoma. Currently available ancillary studies are still of little value in definitively differentiating schwannomas from other choroidal tumors. In the case of atypical findings for a malignant melanoma, a benign neoplasm should be included in the differential diagnosis. This patient avoided enucleation by first having the mass excised. We are unaware of previous reports in which a choroidal schwannoma was diagnosed by surgical excision.

Transsternal maximal thymectomy is effective for extirpation of cervical ectopic thymic tissue in the treatment of myasthenia gravis.

PURPOSE: Extensive extirpation of cervico-mediastinal adipose tissue increases the chance of removing ectopic thymic tissues, thus potentially improving the prognosis of myasthenia gravis after thymectomy. We sought to increase efficacy and safety of transsternal maximal thymectomy (TSMT). MATERIALS AND METHODS: Twenty four patients who underwent TSMT from July 2006 to June 2007 were retrospectively reviewed and compared with 73 patients who underwent transsternal extended thymectomy (TSET) from January 2004 to May 2006. Ectopic thymic tissue in additionally excised cervicomediastinal fat tissue was examined histologically. RESULTS: In TSMT group, operation time, amount of cumulative drainage and duration of drainage were significantly higher than TSET group. However, the difference in hemoglobin count, amount of transfusion, duration of intensive care, postoperative hospital stay, and complication rates were not statistically different. There was no operative mortality in either group. Ectopic thymic tissue was found in 50% of patients. All patients had ectopic thymic tissues in the cervical area. Two patients had additional ectopic tissue in the aortopulmonary window, and 1 patient had ectopic tissue at posterior of the left bracheocephalic vein and lateral of the right phrenic nerve. CONCLUSION: TSMT is more effective in the extirpation of ectopic thymic tissues than TSET without significant impairment of safety, especially in the cervical area.

Primary thyroid lymphoma: role of ultrasound-guided needle biopsy.

OBJECTIVE: The purpose of this study was to describe the sonographic findings of primary thyroid lymphoma and evaluate the role of ultrasound-guided biopsy in diagnosing thyroid lymphoma. METHODS: This study included 6 patients (age range, 56-72 years; mean, 62.5 years). We searched the database of our institution and retrospectively collected data on the thyroid lymphomas that were confirmed pathologically. All of the sonograms and medical records were reviewed retrospectively. RESULTS: All 6 patients had an enlarged neck mass and underwent ultrasound-guided fine-needle aspiration biopsy. The most notable sonographic feature of primary thyroid lymphoma was a marked hypoechoic mass compared with the residual thyroid tissue. Among the 6 patients with a diagnosis of thyroid lymphoma, 3 (50%) had a diagnosis of lymphoma by ultrasound-guided fine-needle aspiration biopsy. Final pathologic results were obtained by ultrasound-guided core needle biopsy (3/6 patients [50%]) or thyroidectomy (3/6 [50%]). Most patients with thyroid lymphoma (5/6 [83.3%]) were found to have diffuse large B-cell lymphoma and were treated with chemotherapy with or without radiotherapy. In 1 patient with follicular lymphoma, diagnosis and treatment were accomplished by total thyroidectomy. CONCLUSIONS: Our results show that ultrasound-guided core needle biopsy can be a safe and accurate method for diagnosing thyroid lymphoma and may be a suitable replacement for diagnostic thyroid surgery.

Sonographically guided core needle biopsy of cervical lymphadenopathy in patients without known malignancy.

OBJECTIVE: The purpose of this study was to retrospectively evaluate the efficacy of sonographically guided core needle biopsy (core biopsy) for diagnosing the causes of cervical lymphadenopathy in patients without known malignancy. METHODS: One hundred fifty-five sonographically guided core biopsies performed in 155 patients with cervical lymphadenopathy were retrospectively evaluated. None of the 155 patients had any known primary malignancy. Final diagnoses were determined by the histologic examination from excision biopsy when performed or by the clinical and sonographic follow-up for more than 12 months. When a lymph node diagnosed as benign by sonographically guided core biopsy regressed spontaneously or by subsequent management, the diagnosis made by the sonographically guided core biopsy was considered correct. When a lymph node diagnosed as benign by sonographically guided core biopsy was unchanged or increased in size with subsequent management, excision biopsy was performed. Diagnostic yield, sensitivity, specificity, accuracy, and complications of core biopsy were evaluated. RESULTS: Histologic diagnosis could be made by sonographically guided core biopsy in 146 (94%) of the 155 patients. The histologic diagnoses were reactive hyperplasia in 44 patients, tuberculosis in 37, Kikuchi disease in 25, metastasis in 16, lymphoma in 16, normal in 7, and toxoplasmosis in 1. Sensitivity, specificity, and accuracy of sonographically guided core biopsy were 97.9%, 99.1%, and 97.9%, respectively. There were no procedure-related complications. CONCLUSIONS: Sonographically guided core biopsy is a safe and efficient tool for diagnosing the cause of cervical lymphadenopathy in patients without known malignancy and may obviate unnecessary excisional biopsy.

Nine-year survival of lymphoblastic lymphoma patients.

This study aimed to analyze the overall survival period of adult lymphoblastic lymphoma patients treated with various therapeutic regimens, and to assess the determinants affecting survival outcome. Twenty-five adult patients with lymphoblastic lymphoma who had been treated at Severance Hospital, Yonsei University College of Medicine, Seoul, Korea from June 1996 to June 2005 were analyzed retrospectively. As an initial remission induction chemotherapy, the hyper-CVAD regimen was performed in eight patients, the Stanford/Northern California Oncology Group (NCOG) regimen in five, the CAVOP regimen in four, the m-BACOP regimen in three, and the CHOP regimen in one patient. Patients were divided into two groups according to their therapeutic modalities. Twenty patients received conventional chemotherapy alone and five received subsequent PBSCT after conventional chemotherapy. Four patients of the PBSCT group underwent autologous PBSCT and one underwent allogeneic PBSCT. The overall response rate was 80% (60% showing a complete response, 20% showing a partial response) and the relapse rate was 73.3%. The overall survival (OS) rate was 55.1% at 1 year, 31.5% at 5 years, and 23.6% at 9 years. The disease-free survival (DFS) rate was 46.7% at 1 year and 30.0% at 7 years. The 5-year OS rate in relation to the regimens was 60% with the Stanford/NCOG regimen, 50% with the CAVOP regimen, and 33.3% with the m-BACOP regimen. The patients treated with the hyper-CVAD regimen had an 18.2% 2-year OS rate, and other patients with CHOP or COPBLAM-V expired early in their course. The OS rate in patients treated with conventional chemotherapy alone was 19.8%, whereas patients treated with subsequent PBSCT after chemotherapy showed 50% overall survival (p=0.25). The age at presentation influenced the outcome of the patients (p=0.01). The Stanford/NCOG regimen is an effective initial choice of therapy for lymphoblastic lymphoma patients, and is superior to the hyper-CVAD regimen in complete response rate and overall survival rate (p =0.36). Addition of PBSCT after chemotherapy may be needed for achieving optimal outcomes.

Focal fibrosis of the breast diagnosed by a sonographically guided core biopsy of nonpalpable lesions: imaging findings and clinical relevance.

OBJECTIVE: The purpose of this study was to evaluate the frequency of focal fibrosis of the breast diagnosed by a sonographically guided core biopsy of nonpalpable lesions, to characterize imaging features, and to evaluate their clinical relevance. METHODS: In a retrospective review of 724 lesions that underwent sonographically guided core biopsy of nonpalpable breast lesions, 62 cases had a diagnosis of focal fibrosis. Two radiologists analyzed the sonographic and mammographic findings according to the Breast Imaging Reporting and Data System. The results were compared with histologic findings at surgery or imaging findings during surveillance. RESULTS: The incidence of focal fibrosis was 8.6% (62/724). Sonographic films were available in 56 cases, so 56 cases were reviewed for their sonographic findings. Among the mammograms reviewed, 64.7% (33/51) had negative findings. Among the sonograms reviewed, the most common features were oval shape (32/56, 57.1%), parallel orientation (36/56, 64.3%), microlobulated margin (24/56, 42.9%), abrupt interface (50/56, 89.3%), isoechoic pattern (42/56, 75.0%), and a lack of posterior acoustic features (45/56, 80.4%). The Breast Imaging Reporting and Data System final assessment was category 3 in 27 (48.2%) and category 4 in 29 (51.8%). Most of the category 4 lesions were category 4A (26/29, 89.7%). Surgical excision (n = 7) and follow-up for at least 1 year (n = 49) showed no malignancy. CONCLUSIONS: Focal fibrosis was found in 8.6% by a sonographically guided core biopsy of nonpalpable breast lesions. Most of the lesions were categorized as probably benign (category 3) or having a low suggestion of malignancy (category 4A). Focal fibrosis diagnosed at core biopsy can be managed with a 6-month follow-up protocol.

Apoptosis and proliferation in paired primary colorectal adenocarcinomas and their liver metastases.

The proliferation potentials and the level of apoptosis were compared in paired primary colorectal adenocarcinomas and their liver metastases within each individual. From a total of 22 patients 44 specimens of paired primary and metastatic tumors were obtained for analysis. The levels of spontaneous apoptosis (a spontaneous apoptosis index, SAI: % apoptotic nuclei among a total of 1000 nuclei) and of proliferation (KI-67 index: % positively stained cells for KI-67 among a total of 1000 cells) were analyzed between primary and metastatic tumors. Survival rates and its relationship with the clinical parameters were also analyzed. The overall survival rate at 5 years was 16.9% with the median survival time of 45 months. T-stage (p=0.005) and time to liver metastasis (synchronous versus metachronous, p=0.03) showed statistical significance in relation to survival. The mean SAI of primary tumors was 1.35 +/- 0.25, which was not statistically different from the 1.58 +/- 0.18 of metastatic tumors (p=0.33). The mean KI-67 indices in primary and metastatic tumors were 23.9 +/- 3.4 and 16.4 +/- 2.5, respectively, and this difference was statistically significant (p=0.016). Subset analysis showed significant difference in the KI-67 index in the synchronous group but not in the metachronous group. No significant difference was shown in the relative ratios of apoptosis to proliferation between the primary tumor and the metastasis within each individual. The results in this study may partly explain the indolent behavior of liver metastasis from colorectal cancer and provides a rationale for the active treatment of metastatic tumors as well as of primary disease.

Low grade gastric mucosa associated lymphoid tissue lymphoma: treatment strategies based on 10 year follow-up.

AIM: To deduce strategic guidelines of gastric mucosa associated lymphoid tissue lymphoma (MALTOMA) by evaluating the long-term outcome of patients in respect to various treatment modalities. METHODS: A total of 55 patients with MALTOMA from May 1992 to August 2002 were retrospectively reviewed. RESULTS: Complete remission was obtained in 24 (82.8%) of 29 patients treated with anti Helicobacter pylori (H pylori) regimen only. The duration to reach complete remission was 12 months (85 percentile, 2-33 months). Five patients showed complete remission with radiation therapy (26-86 months). Two of them were H pylori treatment failure cases. CONCLUSION: H pylori eradication is an effective primary treatment option for low grade MALTOMA and radiation therapy could be considered in patients with no evidence of H pylori infection or who do not respond to H pylori eradication therapy 12 months after successful eradication.