Pulmonary artery sarcoma (PAS) is a rare and fatal disease that often mimics chronic thromboembolic pulmonary hypertension (CTEPH); therefore, diagnosis of PAS is often delayed. Herein, a healthy 74-year-old man was presented with a 4-month history of dyspnea. Chest computed tomography showed wall thickening and stenosis in the main pulmonary artery as well as in both proximal pulmonary arteries. In order to differentiate between unusual CTEPH, vasculitis, and PAS, we performed right heart catheterization and pulmonary angiography. The mean pulmonary arterial pressure was 21 mmHg, and there was severe pulmonary artery stenosis. Thrombi on the pulmonary arterial wall lesions were observed in intravascular ultrasound and optical coherence tomography. Furthermore, the patient had a history of deep vein thrombosis. Therefore, we diagnosed unusual CTEPH. After 6 months of rivaroxaban anticoagulation therapy, a chest X-ray revealed a left lower lobe lung mass, and a positron emission tomography later showed hypermetabolic lesions in the main pulmonary artery wall, in both pulmonary arteries walls, in the lung parenchyma, and in the bones. A biopsy of the right proximal humerus lesion revealed undifferentiated intimal sarcoma. Pulmonary sarcoma is rare, but should be considered when differentially diagnosing main pulmonary artery wall thickening and stenosis. A positron emission tomography may aid in this diagnosis.
Leiomyosarcoma (LMS) of the small intestine is a rare tumor, accounting for about 1% of all malignant mesenchymal lesions in the gastrointestinal tract. Since small bowel tumors are initially asymptomatic and nonspecific, delayed diagnosis and treatment are common. We found that a 44-year-old male patient who came in for lower abdominal pain had partial obstruction in the small bowel. Multiple ascites and ileal tumors involving peritoneal seeding were observed from his abdominal computed tomography. He was diagnosed as epithelioid LMS involving peritoneal transition after surgical resection, and the outpatient department has planned for a conservative therapy with observation. To the best of our knowledge, ileal epithelioid LMS accompanying a huge omental mass, with a size of 18 cm in dimension, and peritoneal seeding has not yet been reported in Korea. We report this rare case with literature review.
Synovial sarcoma is a rare malignancy in the thoracic cavity, especially in the mediastinum. In this paper, a case of primary mediastinal synovial sarcoma is reported. A 34-year-old woman was hospitalized with dyspnea. Her chest X-ray and computed tomography (CT) showed a 16x13x11 cm mass in her anterior mediastinal space. Surgical resection was performed but was incomplete. The pathological and immunohistochemical analysis confirmed the diagnosis of monophasic spindle cell synovial sarcoma. The patient underwent adjuvant radiotherapy for two months, but local recurrence and metastasis occurred in her pleural cavity. She eventually underwent chemotherapy for one year and died 18 months after her operation.
Ha young Lee, Myung Soo Hyun, Kyung Hee Lee, Min Kyoung Kim, Sung Ae Koh, Se Hoon Sohn, Sung Woo Park, Dong Geun Kim, Myung Jin Kim, Hyo Jin Jang, Mi Jin Kim
Yeungnam Univ J Med. 2011;28(1):70-76. Published online June 30, 2011
Rhabdomyosarcomas are soft tissue sarcomas; while extremely rare in adults, they are one of the most common neoplasms in children and adolescents. Histologically, they can be classified into embryonal(ERMS), alveolar(ARMS), pleomorphic, and undifferentiated types. The ARMS type is very rare, and is associated with a poor prognosis. Common primary sites of ARMS are the trunk and extremities. We report on a case of paraaortic, supraclavicular, and axillary lymph node metastasis from paratesticular ARMS treated with VAC(vincristine, dactinomycin, cyclophosphamide)/ IE(ifosfamide, etoposide) chemotherapy in a young adult. Administration of six cycles of chemotherapy with VAC/ IE resulted in complete remission. The patient has maintained complete remission over the past 27 months.
Primary cardiac tumors are very uncommon. Seventy five percent of them are benign tumors and these are mostly myxomas. The malignant cardiac tumors, the majority of which are undifferentiated sarcomas, comprise up to 25% of all cardiac tumors. A primary malignant sarcoma of the myocardium is exceedingly rare. Thus, there have been very few such cases reported in the literature. We present here a case of a 15 year old man who had complaints of orthopnea and increasing exercise intolerance over a one month period. Transthoracic echocardiography demonstrated a well demarcated huge mass with left ventricular inflow obstruction on the posterior wall of the left atrium. The patient's symptoms were relieved by surgery. The histological diagnosis was an unclassified spindle cell sarcoma.
BACKGROUND Accumulating evidence shows that interleukin(IL)-1 plays a critical role in inflammation and connective tissue destruction observed in both osteoarthritis and rheumatoid arthritis. IL-1 induces gene expression related to cytokines, chemokines and matrix metalloproteinases by activation of many different transcription factors. MATERIALS AND METHODS: The chondrosarcoma cell line, SW1353, is known to be a valuable in vitro system for investigating catabolic gene regulation by IL-1beta in chondrocytic cells. To explore and analyze the changes in gene expression by IL-1 responsible for arthritis, SW1353 was treated with IL-1 for 1, 6 and 24 h and then total RNAs were purified for each time. The changes in gene expression were analyzed with 17k human cDNA microarrays and validated by semi-quantitative RT-PCR. RESULTS: Greater than a two-fold change was observed in 1,200 genes including metallothioneins, matrix metalloproteinases, extracellular matrix proteins, antioxidant proteins, cytoskeleton proteins, cell cycle regulatory proteins, proteins for cell growth and apoptosis, signaling proteins and transcription factors. These changes appeared to be correlate with the pathophysiological changes observed in early osteoarthritis. CONCLUSION: cDNA microarray analysis revealed a marked variability in gene expression, and provided insight into the overall molecular changes. The result of this study provide initial information for further studies to identify therapeutic targets in osteoarthritis pathogenesis.
BACKGROUND Doxorubicin has proved to be a useful chemotherapeutic agent especially for osteogenic sarcoma. It induces cancer cell death via apoptosis. MATERIALS AND METHODS: To explore and analyze the changes of gene expression during doxorubicin induced apoptosis on human osteogenic sarcoma, Saos-2 cell, cDNA microarray was performed. After treatment with doxorubicin, total RNA was purified and expressed genes were investigated with a 17k human cDNA microarray. RESULTS: For analysis of the cDNA microarray, the genes were filtered using the sum of the median value of Cy3 and Cy5 signal intensity of greater than 800. Expression of 264 genes was changed by more than 2 fold, and the expression of 35 genes was changed more than 3 fold after treatment with doxorubicin. The genes were primarily related to cell death, cell growth and maintenance, signal transduction, cellular component, transport, and metabolism. CONCLUSION: Treatment with doxorubicin induced expressional change of many genes. Some of the genes might be related with apoptosis directly or indirectly. Further study is now needed to characterize these genes.
Ewing's sarcoma is rarely found occurs in the bones of the hands and feet. We report a case of Ewing's sarcoma of the left calcaneus in a 15-year-old girl who complained of left heel pain and swelling. An open biopsy was performed and histological examination showed the proliferation of uniform small round cells. Immunohistochemical staining for CD99 showed diffuse strong positivity in the cytoplasmic membrane of the tumor cells. After preoperative chemotherapy, a below knee amputation was performed.
Citations
Citations to this article as recorded by
Diagnoses, treatment, and oncologic outcomes in patients with calcaneal malignances: Case series, systematic literature review, and pooled cohort analysis Erik T. Newman, Eveline A. J. van Rein, Nina Theyskens, Marco L. Ferrone, John E. Ready, Kevin A. Raskin, Santiago A. Lozano Calderon Journal of Surgical Oncology.2020; 122(8): 1731. CrossRef
Thallium-201 scintigraphy is used to discriminate the malignant bone tumor from the benign by qualitatively and quantitatively, and to predict the response of preoperative chemotherapy in osteosarcoma, by comparing the changes of thallium uptake ratio after chemotherapy to the tumor necrosis ratio. Thallium-201 scintigraphy scan should be done prior to surgical biopsy. PICKER Prism 2000 gamma camera with high resolution parallel hole collimator is usually used for scanning. The patient is injected with 2-3mCi of Tl-201 and the early phase is checked in 30 minutes and delayed phase in 3 hours. The scan images are visually evaluated by a blinded nuclear medicine physician. We could evaluate true positive, true negative, false positive and false negative by the comparison of results with those of biopsy, and calculate positive and negative predictive value(%), sensitivity(%), specificity(%) and diagnostic accuracy(%). For the quantitative analysis of thallium uptake, we drew the region of interest on the tumor side and contralateral normal side as mirror image, and calculated the uptake ratio with dividing the amount of gamma count in tumor side by normal side. We could calculate the percent changes of thallium uptake ratio in early and delayed phase, and compare them to the ratio of tumor necrosis. Thallium-201 scintigraphy proved as useful imaging study to discriminate malignant bone tumor from benign, but had exception in giant cell tumor and low grade malignant bone tumors. We can use T1-201 scan to differentiate the benign from the malignant tumor, and to evaluate the response of preoperative chemotherapy or radiotherapy, and to determine the residual tumor or local recurrence. For the better result, we need to have a more detail information about false positive cases and a more objective and quantitative reading technique.
Malignant fibrous histiocytoma (MFH) of the spermatic cord represents an uncommon location for the most common soft tissue tumor in adults. MFH of the spermatic cord is extremely rare. No case report has been described in the Korean literature. We report a case of malignant fibrous histiocytoma, myxoid type, arising from left spermatic cord. A 77-year-old male presented with a painless left upper scrotal mass for 5 months. Simple mass excision was performed for a diagnosis. Grossly, the mass closely contacted with the left spermatic cord. Since a myxoid type of malignant fibrous histiocytoma was diagnosed from histopathological findings, left radical orchiectomy with high ligation of the spermatic cord was performed additionally. There were no evidence of local recurrence or metastasis at 6 months after surgery.
Mesenchymal chondrosarcoma is a rare malignant tumor of skeletal and extraskeletal origin, and which shows aggressive local behavior as well as a high metastatic potential. We report 3 cases of mesenchymal chondrosarcoma. Two cases were male and one was female. The ages ranged from 25 to 32 years(mean: 28 years). Tissue was obtained by wide excision in two patients, and incisional biopsy in one. The mass locaterd in the rib(case 1), orbital floor(case 2), and abdominal wall(case 3). Roentgenographically, the tumor resembles ordinary chondrosarcoma, showing osteolytic and obstructive appearance with stippled calcification. Grossly, the tumor was lobulating, solid fish-fleshy like mass with calcification and ossification. Histologically, the tumor shows characteristic bimorphic pattern composed of islands of well differentiated hyaline cartilage admixed with a cellular area of undifferentiated small cells. The small cells usually displayed a hemangiopericytoid or an alveolar pattern.
A rare case of telangiectatic osteosarcoma of the sphenoid bone was reported. The patient was a 27-year-old male and had suffered from left eye protrusion and diplopia for three months. Radiologically, a lobulated osteolytic lesion was located between sphenoid and left temporal bone. Pathologically, the tumor had multiloculated blood filled cystic vascular spaces and osteoid formation by malignant spindle osteoblast cells. The differential diagnosis of this rare entity from aneurysmal bone cyst was important radiologically and pathologically. This case is the second case of telangietatic osteosarcoma of the sphenoid bone followed by a case of Whitehead RE and Melhem ER in 1998.
Clear cell sarcoma is a rare soft tissue sarcoma that occurs tendons and aponeuroses, usually of the lower extremities in young adult. The exact histogenesis is not definitely established, We experienced a case of 58 year-old female who presented with a 3.2x2.2cm sized mass located in the subcutaneous tissue on the left lower thigh. The mass was well circumscribed and grayish firm. Two small satellite nodules were also seen. Histologically the tumor was composed of round to fusiform cells with clear or pale eosinophilic cytoplasm and separated into compact nests or short fascicles by delicated fibrous septa. The melanin pigments and hemosiderin were seen. Tumor cells showed positive reaction for S-100 protein and HMB-45. The ultrastructural examination showed abundant mitochondria and melanosomes.
Sarcoma of the uterus is very rare malignant tumor originating from uterine muscle or connective tissue. We have experienced 20 cases of uterine sarcoma from January 1991 to June 1998. The results were as follows: 1. The pathologic types were 13 cases(65.0%) of leiomyosarcoma, 5 cases(25.0%) of malignant mixed Mullerian tumor, 1 case of rhabdomyosarcoma, and 1 case of angiosarcoma. 2. The average age and parity was 50.2 and 3.7. The chief complaints were irregular vaginal bleeding(35.0%), lower abdominal pain(25.0%), and abdominal mass(25.0%). 3. Nine cases(45.0%) were FIGO stage I, 1 case(5.0%) was stage II, 6 cases(30.0%) were stage III, and 4 cases(20.0%) were stage IV. 4. The survival was from 1.5 months to over 130 months(median 16.5 months), and there was no correlation between survival and FIGO stage or pathologic type. The correlation between survival and number of mitotic figure was incalcurable. 5. CA 125 levels were serially measured as a tumor marker in monitoring patients and the positive rate was 40%. Further study was needed to make a conclusion for usefulness of CA 125 as a tumor marker.
Pure primary hepatic rhabdomyosarcoma in adult is very uncommon. There have been only five previous case of primary rhabdomyosarcoma of the adult liver. A case of hepatic ihabdomyosarcoma was diagnosed in a 52 year-old female. She was admitted to the hospital due to the epigastric pain and weight loss. A CT scan of the abdomen showed a large hypodense mass with focal calcification occupies most of the both lobes of the liver. The liver biopsy showed massive liver tumor composed entirely of oval shaped cells showing light microscopic and immunohistochemical evidence of rhabdomyoblastic differentiation. We report a case of hepatic rhabdomyosarcoma with review of literature.