The Clinical Outcome of Extraskeletal Myxoid Chondrosarcoma / 대한정형외과학회잡지

Purpose@#Extraskeletal myxoid chondrosarcoma (EMC) is an extremely rare malignant mesenchymal neoplasm, accounting for less than 3% of soft tissue sarcomas. This sarcoma is usually characterized by its indolent course. This study examined the clinical manifestations and oncologic outcomes of EMC. @*Materials and Methods@#Seventeen patients diagnosed and treated for EMC between January 2008 and December 2018 were enrolled in this study. The cohort was then reviewed regarding age, gender, symptom onset, tumor location, magnetic resonance images, surgical margin, and pathologic diagnosis. The time to local recurrence and metastasis, follow-up duration, and the patients’ final status were analyzed. @*Results@#The patients were comprised of 10 males and seven female patients with a mean age of 54 (range, 31–79). The tumor location was the buttock in five, thigh in four, knee in three, foot in three, shoulder in one, and back in one. The average tumor diameter was 11.5 cm (range, 2.2–23.2 cm). At the time of diagnosis, five patients were American Joint Committee on Cancer stage II; three were IIIA; three were IIIB; six were IV. Local recurrence occurred in 12 cases, and distant metastasis occurred in 15 cases. The five-year overall survival of the patients with EMC was 73%±17%, and two patients died of the disease. @*Conclusion@#Despite the high rate of local recurrence and distant metastasis, the long-term survival rate in patients with EMC is quite high because of its indolent characteristics.

The Clinical Outcome of Dedifferentiated Chondrosarcoma / 대한정형외과학회잡지

PURPOSE: A dedifferentiated chondrosarcoma is a rare lethal tumor characterized by a low grade chondrosarcoma juxtaposed with a high grade dedifferentiated sarcoma, such as osteosarcoma, fibrosarcoma. The aim of our study was to document the clinical manifestation and oncologic outcomes of a dedifferentiated chondrosarcoma. MATERIALS AND METHODS: This study identified 11 patients who were diagnosed and treated for dedifferentiated chondrosarcoma between January 2007 and December 2016. The identified cohort was then reviewed regarding age, sex, symptom onset, tumor location, magnetic resonance imagings (MRIs), surgical margin, and pathologic diagnosis. The time to local recurrence and/or metastasis, follow-up duration, and the patients' final status were analyzed. RESULTS: The patients were comprised of 7 males and 4 females with a mean age of 54 years (range, 33–80 years). The location of the tumor was in the femur in 6 cases, pelvis in 4 cases, and metatarsal in 1 case. The average tumor diameter was 12.7 cm (range, 6.0–26.1 cm). At the time of diagnosis, 2 patients showed pathologic fracture; 1 patient was Enecking stage IIA, 9 patients were stage IIB, and 1 patient was stage III. Eight patients were classified as a primary dedifferentiated chondrosarcoma and 3 patients were secondary. One of the primary lesions was misinterpreted initially as a low grade chondroid lesion by MRI and underwent curettage. Local recurrence occurred in 8 cases and distant metastasis occurred in 10 cases with a mean duration of 8 months (range, 2–23 months) and 7 months (range, 1–32 months), respectively. The three-year overall survival of patients with dedifferentiated chondrosarcoma was 18%, and 10 patients died due to disease progression. CONCLUSION: Dedifferentiated chondrosarcoma developed lung metastases in the early period of the clinical courses and the prognosis was dismal.

Factors that Predict Clinical Benefit of EGFR TKI Therapy in Patients with EGFR Wild-Type Lung Adenocarcinoma / 결핵및호흡기질환

BACKGROUND: Epidermal growth factor receptor (EGFR) mutations in non-small cell lung cancers have emerged as key predictive biomarkers in EGFR tyrosine kinase inhibitor (TKI) treatment. However, a few patients with wild-type EGFR also respond to EGFR TKIs. This study investigated the factors predicting successful EGFR TKI treatment in lung adenocarcinoma patients with wild-type EGFR. METHODS: We examined 66 patients diagnosed with lung adenocarcinoma carrying wide-type EGFR who were treated with EGFR TKIs. The EGFR gene copy number was assessed by silver in situ hybridization (SISH). We evaluated the clinical factors and EGFR gene copy numbers that are associated with a favorable clinical response to EGFR TKIs. RESULTS: The objective response rate was 12.1%, while the disease control rate was 40.9%. EGFR SISH analysis was feasible in 23 cases. Twelve patients tested EGFR SISH-positive, and 11 were EGFR SISH-negative, with no significant difference in tumor response and survival between EGFR SISH-positive and -negative patients. The overall median progression-free survival (PFS) and overall survival (OS) of 66 patients were 2.1 months and 9.7 months, respectively. Female sex and Eastern Cooperative Oncology Group (ECOG) performance status (PS) of 0–1 were independent predictors of PFS. ECOG PS 0–1 and a low tumor burden of extrathoracic metastasis were independent predictors of good OS. CONCLUSION: Factors such as good PS, female sex, and low tumor burden may predict favorable outcomes following EGFR TKI therapy in patients with EGFR wild-type lung adenocarcinoma. However, EGFR gene copy number was not predictive of survival.

Cytological Features That Differentiate Follicular Neoplasm from Mimicking Lesions

BACKGROUND: It is difficult to correctly diagnose follicular neoplasms (FNs) on fine-needle aspiration cytology (FNAC) because it shares many cytological features with other mimicking lesions. The aim of this study was to identify the cytological features that differentiate FNs from mimicking lesions. METHODS: We included the cytological slides from 116 cases of thyroid FN diagnosed on FNAC, and included their subsequent histological diagnoses. We evaluated the cytological architectural pattern and nuclear features of the lesions according to their histological groups. RESULTS: The final histological diagnoses of the 116 cases varied, and included 51 FNs (44%), 47 papillary thyroid carcinomas (40%) including follicular variant, and seventeen cellular nodular hyperplasias (15%). Regardless of the final histological diagnosis, microfollicular pattern was observed in most cases. On the other hand, trabecular pattern was identified in 34% of FNs, but not in any other lesions. Additionally, elongated nuclei and ground glass chromatin were found in only some papillary thyroid carcinomas. CONCLUSIONS: This study shows that the trabecular pattern is a representative cytological feature of FNs that can be used to distinguish FNs from mimicking lesions. In addition, nuclear shape and chromatin pattern can be used to further confirm the diagnosis of FNs from mimicking lesions through FNAC.

The Significance of TROP2 Expression in Predicting BRAF Mutations in Papillary Thyroid Carcinoma

BACKGROUND: Trophoblast antigen 2 (TROP2) is a human trophoblast cell-surface glycoprotein that is overexpressed in several types of epithelial cancers, and is suggested to be associated with an unfavorable prognosis. BRAF mutations are the most common genetic alteration in papillary thyroid carcinoma (PTC). We evaluated the correlation between TROP2 expression and BRAF mutation in PTC. METHODS: First, we carried out pyrosequencing for BRAF mutations and immunohistochemistry for TROP2 expression with a tissue microarray consisting of 52 PTC cases. Membranous staining in at least 5% of tumor cells was designated as positive staining and we analyzed the relationship between TROP2 expression and diverse clinicopathological factors, including BRAF mutation. Second, we tested TROP2 mRNA expression in three thyroid cancer cell lines with BRAF mutations (BCPAP, SNU790, and 8505C) and a normal thyroid cell line. Additionally, we checked TROP2 protein levels in a normal thyroid cell line after introduction of the BRAF V600E mutation. RESULTS: In this study, 21 of 26 cases with BRAF mutation showed TROP2 immunoreactivity, whereas all 26 cases without BRAF mutation showed no immunoreactivity for TROP2 with a statistically significant difference (p<.001). Upregulation of TROP2 mRNA was observed in all three thyroid cancer cell lines, but not in the normal thyroid cell line. Interestingly, however, the TROP2 expression was increased in the normal thyroid cell line after introduction of the BRAF V600E mutation. CONCLUSIONS: Based on these results, we concluded that TROP2 expression is significantly associated with BRAF mutation and that TROP2 immunohistochemistry could be used for predicting BRAF mutations or diagnosing papillary thyroid carcinoma.

Importance of Individual Ghost Cells in Fine-Needle Aspiration Cytology Diagnosis of Pilomatricoma

BACKGROUND: Although histological diagnosis of pilomatricoma is not difficult because of its unique histological features, cytological diagnosis through fine-needle aspiration cytology (FNAC) is often problematic due to misdiagnoses as malignancy. METHODS: We reviewed the cytological features of 14 cases of histologically-proven pilomatricoma from Korea Cancer Center Hospital, with a discussion on the diagnostic pitfalls of FNAC. RESULTS: Among 14 cases of pilomatricoma, 10 (71.4%) were correctly diagnosed through FNAC, and two (14.3%) were misdiagnosed as carcinoma. Cytologically, all cases had easily recognizable clusters of basaloid cells and foreign body-type multinucleated cells. Although ghost cells were also found in all cases, some were inconspicuous and hardly recognizable due to their small numbers. CONCLUSIONS: An accurate diagnosis of pilomatricoma in FNAC is feasible with consideration of clinical information and close examination of ghost cells.

Clear Cell Chondrosarcoma Mimicking Benign Bone Tumors / 대한정형외과학회잡지

PURPOSE: Clear cell chondrosarcoma may have a benign appearance even on a magnetic resonance imaging (MRI). Hence, it can be confused with benign bone tumors, such as a giant cell tumor or chondroblastoma. The aim of our study was to document the doctorassociated diagnostic errors in patients with clear cell chondrosarcoma and oncologic outcomes of these lesions, which were misdiagnosed as benign bone tumors. MATERIALS AND METHODS: We identified 10 patients who were diagnosed with and treated for clear cell chondrosarcoma between January 1996 and December 2014. One patient was excluded due to insufficient clinical data. We then reviewed their data regarding age, gender, symptom onset, tumor location, initial imaging diagnosis, and associated previous treatment. We examined the errors of surgeons and pathologists with respect to patient and tumor characteristics. We also analyzed treatment delay, time to local recurrence, metastasis, follow-up duration, and the oncologic outcome. RESULTS: The initial presumptive diagnosis based on MRIs for all 9 patients was benign bone tumor. Among 8 patients who underwent inappropriate procedure, half of them were diagnosed as clear cell chondrosarcoma immediately after the curettage. As for the remaining 4 patients, the surgeon did not send any tissue samples to a pathologist for a definite diagnosis in three patients and a pathologist made an incorrect diagnosis in one patient. We performed an appropriate surgery on all patients with a wide surgical margin. The average treatment delay was 27 months (range, 0–127 months), and the average follow-up duration was 65 months (range, 13–164 months). One patient had local recurrence after 12 months. Metastatic disease developed in 2 patients with a median time to definitive treatment of 24 months (12–37 months). Ten-year overall survival of patients with clear cell chondrosarcoma was 78%, and two patients died due to disease progression. CONCLUSION: Misdiagnosis of clear cell chondrosacroma as a benign bone tumor is not uncommon, even for experienced orthopaedic oncologists, resulting in definite curative surgery without biopsy. An inappropriate primary treatment may increase the risk of local recurrence and metastasis. Therefore, a proper subsequent surgery is mandatory for patients with clear cell chondrosarcoma who received inadvertent curettage.

The Role of Notch1 Signaling in Anaplastic Thyroid Carcinoma / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: The Notch signaling pathway is widely expressed in normal, reactive, and neoplastic tissues; however, its role in thyroid tissues has not been fully elucidated. Therefore, this study was conducted to characterize the expression of the Notch signaling pathway in papillary thyroid cancer (PTC) cells and anaplastic thyroid cancer (ATC) cells. MATERIALS AND METHODS: Expression of activated Notch1 in ATC and PTC paraffin-embedded tissues was determined by immunohistochemistry. The small interfering RNA techniquewas employed to knock down Notch1 expression in ATC and PTC cell lines. RESULTS: The expression of activated Notch1 was higher in ATC cases than in PTC cases. Inhibition of Notch1 significantly reduced proliferation and migration of ATC cells, but not PTC cells. In addition, inhibition of Notch1 in ATC cells significantly reduced the expression of key markers of epithelial-mesenchymal transition and cancer stem cells. Conversely, changes in the expression of these proteins were not observed in PTC cells. CONCLUSION: The results of this study suggest that Notch1 expression plays different roles in tumor progression in ATC and PTC cells. We also found that Notch1 expression was significantly related to the highly invasive or proliferative activity of ATC cells.

The Clinical Outcome of Soft Tissue Solitary Fibrous Tumor / 대한정형외과학회잡지

PURPOSE: Solitary fibrous tumors (SFT) represent a rare entity of soft tissue tumors which are exclusively located in the thoracic cavity as pleural fibrous tumors. Extrathoracic SFT in the soft tissues are very rare. We analyzed the oncologic outcome of the soft tissue SFT. MATERIALS AND METHODS: Between January 2009 and December 2014, we treated 6 soft tissue SFT cases. Patients' clinical data, magnetic resonance imagings and pathologic findings were reviewed. RESULTS: The patients included two women and four men. The average age of the patients was 45 years (range, 32-56 years). The mean tumor size is 7.2 cm (range, 3.5-13.0 cm). One patient received wide excision of the tumor and remaining 5 patients marginal excision. After the excision, pathologic report confirmed that 4 patients showed margin positive. However, no patient received further surgery or adjuvant treatments. After the immunohistochemistry, 2 out of 6 patients identified as malignant soft tissue SFT. The mean follow-up period was 25.8 months (range, 6-66 months). Although 4 patients revealed margin positivity, no subsequent local recurrence or distant metastasis was observed. CONCLUSION: Since the SFT are categorized as intermediated biological potential with a low risk of metastasis and recurrence, marginal excision can be a one of the surgical options for these tumors which are located adjacent to vital structures. However, close observation is needed because local recurrence can be significantly higher the patients with positive resection margins.

Durable Response to Pazopanib in a Patient with Metastatic Alveolar Soft Part Sarcoma / 이화의대지

Alveolar soft part sarcoma (ASPS) is a rare form of soft tissue sarcoma, and frequently, metastases are found at diagnosis. In patients with metastatic or unresected ASPS, systemic treatment is extremely limited, because conventional chemotherapeutic agents have not been effective in most cases. A novel agent inhibiting angiogenesis, pazopanib, has been proven to be effective for metastatic soft tissue sarcoma in a second-line setting. However, the efficacy of pazopanib in ASPS has not yet been reported. A 22-year-old man presented with right calf ASPS and multiple lung metastases. Pazopanib as a second-line treatment showed significant tumor response. To the best of our knowledge, this is the first report of the effectiveness of pazopanib in ASPS.

Giant Cell-Rich Osteosarcoma: A Tumor Simulating Borderline Lesion / 대한정형외과학회잡지

PURPOSE: Giantcell-rich osteosarcoma (GCRO) is a rare subtype of osteosarcoma. We reviewed; 1) radiological finding of GCRO and clinical impression-related diagnostic workup at referral center, 2) diagnostic delay until a proper diagnosis is made, 3) impact of diagnostic delay on the oncologic outcome. MATERIALS AND METHODS: We reviewed 17 patients with GCRO. We investigated the plain radiographic finding, tumor size and location, presence of pathologic fracture, clinical impression and pathological diagnosis at referral center, diagnostic delay, definitive treatment, local recurrence, metastasis, and survival rate. RESULTS: Eleven cases (64.7%) showed a plain radiographically, purely osteolytic pattern while 6 cases (35.3%) showed mixed osteolytic and sclerotic lesion. Diagnosis at primary center was osteosarcoma in 7 (41.2%), giant cell tumor in 7 (41.2%), and benign bone tumor in 3 (17.6%). Six patients (35.3%) experienced diagnostic delay. Mean diagnostic delay was 3.1 months (1 to 8). At final follow-up 5-year actuarial survival rate of 17 patients was 65%+/-25%. Although 11 patients without diagnostic delay showed a tendency of high survival over 6 patients with diagnostic problem, there was no statistical significance (p=0.14). CONCLUSION: GCRO is a rare subtype of osteosarcoma simulating giant cell tumor both pathologically and radiologically. Careful diagnostic approach is required in order not to misdiagnose this malignant tumor.

Clinical implications of proliferation activity in T1 or T2 male gastric cancer patients

Proliferation activity has already been established as a prognostic marker or as a marker for anticancer drug sensitivity. In gastric cancer, however, the prognostic significance of proliferation activity is still being debated. Several studies evaluating proliferation activity using Ki-67 have shown controversial results in terms of the relationship between proliferation activity and overall survival (OS) or drug sensitivity in gastric cancer patients. Because cytoskeleton-associated protein 2 (CKAP2) staining has recently been introduced as a marker of proliferation activity, we analyzed 437 gastric cancer tissues through CKAP2 immunohistochemistry, and we evaluated the chromatin CKAP2-positive cell count (CPCC) for proliferation activity. Although the CPCC did not show any significant correlation with OS in the male, female or total number of cases, it did show a significant correlation in the T1 or T2 male patient subgroup, according to log-rank tests (P=0.001) and univariate analysis (P=0.045). Additionally, multivariate analysis with the Cox proportional hazard regression model showed a significant correlation between the CPCC and OS (P=0.039) for the co-variables of age, gender, T stage, N stage, histology, tumor location, tumor size and adjuvant chemotherapy. In male gastric cancer cell lines, faster-growing cancer cells showed higher sensitivity to cisplatin than slow-growing cells. Thus our study indicates that CPCC-measured proliferation activity demonstrates a significantly worse prognosis in T1 or T2 male gastric cancer patients. The CPCC will help to more precisely classify gastric cancer patients and to select excellent candidates for adjuvant chemotherapy, which in turn will facilitate further clinical chemotherapeutic trials.

Rosai-Dorfman Disease as a Solitary Lesion of the Tibia / 대한골관절종양학회지

Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder of lymph node and extranodal site. Bone involvement is very rare. We report a case of extranodal RDD of the tibia in 32-year old male. The patient presented with pain with no evidence of lymphadenopathy. Clinico-radiologic diagnosis was metastatic carcinoma or Langerhans cell histiocytosis, but, histopathologic examination confirmed the diagnosis with RDD. We performed curettage on the osteolytic lesion of tibia. In South Korea, there was no report about RDD of the extremity and we want to report this case with review of the literature.

Serial Serum HER2 Measurements for the Detection of Breast Cancer Recurrence in HER2-Positive Patients / 한국유방암학회지

PURPOSE: The measurement of serum human epidermal growth factor receptor 2 (HER2) extracellular domain levels is a well-established method for evaluating whether a metastatic HER2-positive breast cancer patient will respond to HER2-targeted treatment. However, little is known about the value of serum HER2 for detecting disease relapse following curative surgical treatment in breast cancer patients. The purpose of this study was to evaluate the sensitivity of serum HER2, carcinoembryonic antigen (CEA), and carcinoma antigen 15-3 (CA 15-3) for the detection of disease recurrence in postoperative breast cancer patients with a primary HER2-positive tumor. METHODS: Serial measurements were taken of serum HER2, CEA, and CA 15-3 levels in patients with primary invasive HER2-positive breast cancer who underwent curative surgical treatment between January 2008 and December 2010. Following treatment, serum HER2 levels were monitored every 6 months using a chemiluminescence immunoassay. RESULTS: Overall, 264 patients were analyzed in this retrospective study. The median follow-up period was 27.7 months, and 24 patients relapsed during follow-up. The sensitivity of serum HER2, CEA, and CA 15-3 for the detection of disease recurrence was 37.5%, 25.1%, and 12.5%, respectively. Sensitivity increased to 45.8% when all three tumor markers were combined in the analysis. In a subgroup of patients without liver disease, the sensitivity of serum HER2, CEA, and CA 15-3 was 57.1%, 21.4%, and 14.3%, respectively. Of the 264 patients in this study, 80 patients had chronic hepatitis, liver cirrhosis, or abnormal aspartate aminotransferase or alanine aminotransferase levels during the follow-up period. Following the exclusion of these patients, the sensitivity of serum HER2 for the detection of disease recurrence increased to 57.1%. CONCLUSION: Serial serum HER2 measurement may be useful for the detection of disease relapse in patients with HER2-positive breast cancer. Abnormal liver function can result in elevated serum HER2 in the absence of disease recurrence.

Diagnosis, Treatment and Prognosis of Low Grade Central Osteosarcoma / 대한골관절종양학회지

PURPOSE: We analyzed the diagnosis and the treatment outcomes of patients with central low grade osteosarcoma. MATERIALS AND METHODS: We retrospectively reviewed 16 patients with central low grade osteosarcoma were treated at out institution between 1994 and 2011. RESULTS: There were 4 men and 12 women with mean age of 26 years. Eleven patients were correctly diagnosed but 5 patients were misdiagnosed as osteoid osteoma, non ossifying fibroma, aneurysmal bone cyst, desmoplastic fibroma. 15 patients finally received wide margin en bloc excision and one of them treated under neoadjuvant chemotherapy. Final survival status was continuous disease free in 14 and 1 patient died of renal cell cancer. Remaining 1 with multifocal lesions is alive with disease for 7 years only treated radiation therapy on residual tumors. Nine (56%) of 16 tumors showed extra-osseous extension of tumor (56%) and 1 of them showed extra-compartmental tumors. CONCLUSION: The diagnosis of central low grade osteosarcoma is challenging, however, considering of the clinical suspicion, the typical findings of radiologic and pathologic features, proper diagnosis is needed. This tumor should be treated with wide excision, even after an intralesional excision, to avoid local recurrence or transformation to higher histologic grade.

Soft Tissue Malignant Myoepithelioma in the Extremities / 대한골관절종양학회지

PURPOSE: We report the diagnosis, treatment outcomes and prognosis of the patients with soft tissue malignant myoepithelioma in the extremities. MATERIALS AND METHODS: We retrospectively reviewed 6 patients with soft tissue malignant myoepithelioma in the extremities who were treated at our institution between 2008 and 2014. Two patients received unplanned excision at another hospital and remaining 4 patients underwent the biopsy procedures and received wide excision at our hospital. RESULTS: There were 3 men and 3 women with mean age of 41 (33-54) years. The average follow up was 28 (9-45) months. Among the 6 patients, only 4 patients underwent biopsy procedures under the impression of malignant soft tissue sarcoma. Surgical margins for these 4 patients were negative. Two patients who had unplanned excision received another re-excision and one of them showed no residual tumor in the resected specimen. Local recurrences were developed in all patients and distant metastasis in 4 patients. All 4 patients who developed distant metastasis died due to disease progression. Among the 2 patients who developed local recurrence only, one patient has another local recurrence after re-operation and remaining one patient is no evidence of disease for 2 years after resection of locally recurred mass. CONCLUSION: Soft tissue malignant myoepithelioma in the extremities is a rare disease and shows an aggressive behavior. Appropriate biopsy under the impression of soft tissue malignancy is necessary and complete surgical resection with wide margins is the recommended treatment of choice.

Fine Needle Aspiration Cytology of Parathyroid Lesions

BACKGROUND: There has been an increase in the use of fine needle aspiration cytology (FNAC) for the diagnosis of parathyroid lesions (PLs). Differentiation between a thyroid lesion and a PL is not easy because of their similar features. We reviewed parathyroid aspirates in our institution and aimed to uncover trends in diagnostic criteria. METHODS: We selected 25 parathyroid aspirates (from 6 men and 19 women) confirmed surgically or immunohistochemically from 2006 to 2011. RESULTS: Major architectural findings of PLs include scattered naked nuclei, loose clusters, a papillary pattern with a fibrovascular core, tight clusters, and a follicular pattern. These architectures were commonly admixed with one another. Cytological features included anisokaryosis, stippled chromatin, a well-defined cell border, and oxyphilic cytoplasm. Eighteen of the 25 patients were diagnosed with PL using FNAC. Seven patients had been misdiagnosed with atypical cells (n=2), benign follicular cells (n=2), adenomatous goiter (n=2) and metastatic carcinoma (n=1) in FNAC. Using clinicoradiologic data, the sensitivity of the cytological diagnosis was 86.7%. The cytological sensitivity decreased to 50% without this information. CONCLUSIONS: FNAC of PL is easily confused with thyroid lesions. A combination of cytological parameters and clinical data will be required to improve the diagnostic sensitivity of PLs.

High-dose chemotherapy and autologous peripheral blood stem cell transplantation in the treatment of children and adolescents with Ewing sarcoma family of tumors / 소아과

PURPOSE: We performed a pilot study to determine the benefit of high-dose chemotherapy and autologous peripheral blood stem cell transplantation (HDCT/autoPBSCT) for patients with Ewing sarcoma family of tumors. METHODS: We retrospectively analyzed the data of patients who received HDCT/autoPBSCT at Korea Cancer Center Hospital. Patients with relapsed, metastatic, or centrally located tumors were eligible for the study. RESULTS: A total of 9 patients (3 male, 6 female), with a median age at HDCT/autoPBSCT of 13.4 years (range, 7.1 to 28.2 years), were included in this study. Patients underwent conventional chemotherapy and local control either by surgery or radiation therapy, and had achieved complete response (CR, n=7), partial response (n=1), or stable disease (n=1) prior to HDCT/autoPBSCT. There was no transplant-related mortality. However, the median duration of overall survival and event-free survival after HDCT/autoPBSCT were 13.3 months (range, 5.3 to 44.5 months) and 6.2 months (range, 2.1 to 44.5 months), respectively. At present, 4 patients are alive and 5 patients who experienced adverse events (2 metastasis, 2 local recur, and 1 progressive disease) survived for a median time of 2.8 months (range, 0.1 to 10.7 months). The 2-year survival after HDCT/autoPBSCT was 44.4%+/-16.6% and disease status at the time of HDCT/autoPBSCT tended to influence survival (57.1%+/-18.7% of cases with CR vs. 0% of cases with non-CR, P=0.07). CONCLUSION: Disease status at HDCT/autoPBSCT tended to influence survival. Further studies are necessary to define the role of HDCT/autoPBSCT and to identify subgroup of patients who might benefit from this investigational treatment.

Treatment Outcomes of Sacral Giant Cell Tumor / 대한골관절종양학회지

PURPOSE: We analyzed the treatment outcomes of patients with sacral giant cell tumor. MATERIALS AND METHODS: We retrospectively reviewed 7 patients with giant cell tumor of the sacrum who were treated at out institution between 1990 and 2012. RESULTS: There were 2 men and 5 women with mean age of 23.6 years. The average follow up was 52.3 months (range, 15-73 months). Six patients received surgical treatment. Intralesional curettage was performed for the 5 patients and marginal resection for another one patient. The remaining one patient was received radiation only. The patients who received radiation therapy and marginal excision had no residual or recurrent tumors. Of 5 patients with intra-lesional excision, one patient needs one more operation; two patients need two more operation for local control of the giant cell tumor. The remaining two patients failed to gain local control in spite of additional treatments. CONCLUSION: For the treatment of sacral giant cell tumor, intralesional resection can be one of the treatments option with minimal neurologic injury. Furthermore, radiation therapy can be recommended when complete excision or curettage is impractical.

Inflammatory Myofibroblastic Tumor of Extremities / 대한골관절종양학회지

PURPOSE: We analyzed the oncologic characteristics and outcome of patients with inflammatory myofibroblastic tumor of extremities. MATERIALS AND METHODS: Among the soft tissue tumor patients who were treated between 1999 and 2012, 5 patients who were pathologically confirmed as the inflammatory myofibroblastic tumor of extremities were analyzed retrospectively. RESULTS: There were 1 man and 4 women with mean age of 44 years (37-55 years). The average follow up was 34.6 months (8-87 months). All patients underwent surgical treatment. Only 1 patient had wide resection margin and remaining 4 had marginal (3) or intralesional (1) resection margin. All of 4 patients without wide resection margin developed local recurrence at 10.3 months (8-19 months). Malignant transformation to fibrosarcoma was occurred in 2 patients who developed local recurrence, and 1 patient developed multiple metastases to lung, liver and lymph nodes and expired at 37 months. Three of 5 patients had tumor location abutted to or invasion to major arteries and 1 patient had tumor invading sciatic nerve. CONCLUSION: It is observed that inflammatory myofibroblastic tumor of extremities is usually located near the major neurovascular structure. Wide resection should be considered as the initial surgical treatment because this tumor showed a high local recurrence rate and possibility of malignant transformation.