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Extensive chest wall resection carries the risk of difficult reconstruction and surgical complications. We report our experience on chest wall reconstruction using titanium plates for a wide thoracic defect after tumor resection. A 74-year-old man was diagnosed with chondrosarcoma of the 6th rib on the right. He needed extensive chest wall resection because of skip lesions on 4th rib noted on operative inspection, leaving a defect measuring 33 × 20 cm. Reconstruction using 5 transverse titanium plates sandwiched between an expanded polytetrafluoroethylene patch and a polypropylene mesh sheet stabilized the chest wall. This reconstruction allowed successful separation from ventilatory support after operation. The postoperative course was uneventful, and he was discharged on postoperative day 20. The advantages of this form of reconstruction over conventional prostheses are rigidity, and stability and usability.

A 57-year-old woman was admitted with chief complaints of pain in the right lower quadrant and weight loss. Contrast computed tomography of the abdomen revealed a tumor in the right inferior aspect of the liver. The patient was diagnosed with cystic renal cell carcinoma or lymphangioma, and she underwent surgery. The tumor was approached via upper abdominal midline incision and was found to be immobile. The tumor had adhered tightly to the right 12th rib and was resected along with the 12th rib. Gross pathology of the resected tissue revealed a jelly-like component inside, and the patient was histopathologically diagnosed with chondrosarcoma (grade 1). The patient is currently undergoing outpatient follow-up; a 1-year postoperative follow-up did not show any recurrence or metastasis. Chondrosarcoma accounts for approximately 20% of primary malignant bone tumors. It has a low response rate to both chemotherapy and radiotherapy, and surgical treatment remains the most effective method. Bone tumors must be considered in cases of atypical retroperitoneal tumors in which a definitive diagnosis cannot be made using diagnostic imaging.

A 52-year-old woman was admitted to the hospital because of abnormal shadow of the left lung. Chest computed tomography (CT) showed a 10.0 x 6.6 cm mass arising from the left chest wall. The diagnosis of grade II chondrosarcoma was made with CT guided percutaneous needle biopsy. At operation, the irregularly-shaped tumor was arising from the 7th rib. Chest wall resection and partial resection of the left lower lobe including the tumor was performed. Multiple miliary disseminations were noted on the pleura and complete removal of these miliary lesions was impossible. Adjuvant chemotherapy using OK-432, carboplatin and etoposide was tried as treatment for pleural dissemination. No signs of disease progression have been seen as of 12 months after the operation. Radical excision of chondrosarcoma of the rib including all pleural dissemination is difficult. Multidisciplinary approach including resection of a primary tumor has potential to improve prognosis in some cell types. And long term follow-up is necessary to determine effectiveness of adjuvant chemotherapy in this case.

A 62-year-old man was referred to our hospital with a 10 cm retroperitoneal tumor, which was incidentally found on the abdominal computed tomography (CT). CT-guided percutaneous needle biopsy revealed that the tumor was malignant, but could not define the exact pathology of the tumor. The tumor was resected. The pathological study revealed atypical spindle to polygonal shaped cells embedded in the myxomatous stroma. The tumor was diagnosed as extraskeletal myxoid chondrosarcoma. Walking difficulty was observed postoperatively, which was considered to have been caused by damage of a branch of the femoral nerve. The difficulty had gradually overcome with rehabilitation before discharge.

We experienced perioperative management for excision of a huge sternal chondrosarcoma squeezing the heart. A 46-year-old woman could not sleep due to dyspnea for 6 months. Dyspnea increased in the right decubitus and disappeared in the left decubitus. This suggested that the heart was squeezing the tumor. MRI and echocardiography revealed no invasion to the heart. Surgical removal was proposed. Anesthesia was induced with propofol and fentanyl. As this tumor composed of bone-like tissue and was tightly connected with thoracic cage, we thought that the use of muscle relaxant at the induction of anesthesia would not cause cardiovascular collapse and ventilation insufficiency even if the tumor was huge. Vecuronium was administered to facilitate endotracheal intubation. Hemodynamic variables were stable and mask ventilation was uneventful. Anesthesia was maintained with propofol, sevoflurane, supplemental dose of fentanyl and epidural anesthesia. The tumor had invaded the myocardium and was successfully flaked off from the myocardium without using any assisted devices. The tumor was excised including thoracic wall (sternum and parts of 3rd to 12th ribs, 20 cm in diameter). Thoracoplasty was performed using Marlex Mesh. Muscle relaxant was reversed after the end of the operation. Respiration was stable without flail chest. Endotracheal tube was successfully removed in the operation room. The postoperative course was uneventful.

The patient was a 47-year-old man who had visited a nearby physician with a chief complaint of bloody sputum. This patient underwent partial resection of the left upper lobe including a tumor adjacent to a huge bulla revealed by a plain chest radiograph. The resected tumor was composed of adenocarcinomatous and sarcomatous components, including partially-differentiated chondrosarcoma and rhabdomyosarcoma. The diagnosis was therefore true pulmonary carcinosarcoma. The sarcoma component recurred in the remaining part of the left lung. This recurrent lesion was surgically unresectable, but was regionally controlled with radiotherapy and bronchial arterial infusion of anticancer agents. However, distant metastases occurred to the brain, liver, and small intestine, and digestive tract bleeding also occurred. Nevertheless, chemotherapy, linac radiosurgery, percutaneous radiofrequency ablation, and partial small intestinal resection combined with rectal resection were performed. At autopsy, a metastatic lesion was also detected in the pancreas. Liver metastatic lesions were determined to be the adenocarcinomatous component, and the other recurrent or metastatic lesions, except for those in the brain, were all composed of poorly-differentiated sarcomatous tissue.

A 66-year-old man was admitted to our hospital because of induction therapy of interferon for chronic hepatitis due to hepatitis C virus. On routine investigation, a mass-like lesion was detected at the level of the hilum of the left lung on the chest plain radiograph. On the thoracic CT, the heterogeneous tumor, including calcification, was 6 cm in diameter in the posterior mediastinal area, and the rib and vertebra had been damaged by it. The tumor extended to the descending aorta and the left main bronchus. A small nodule in the left S1 + 2 was noted on another slice section of this thoracic CT. The possible diagnoses, based on these radiological findings, for this tumor were primary lung cancer, posterior mediastinal tumor and malignant tumor originating from the chest wall. The diagnosis of chondrosarcoma was made based on the histological findings of the specimen obtained from the tumor. This tumor could not be resected in this case, because it had extensively damaged the rib and vertebra, and the nodular lesion in the left S1 + 2 was regarded as a metastasis from it. Although reports of chondrosarcoma originating from a rib and presenting as a posterior mediastinal tumor-like shadow are rare, chondrosarcoma should be considered as a possible diagnosis for such tumors-posterior mediastinal masses with characteristic findings such as a large tumor in contact with the chest wall, and containing calcification, with destruction of bone and dissection of the rib as shown in our CT findings.

The patient was a 78-year-old man with gross hematuria. A non-papillary and non-pedunculated tumor was found on the posterior wall of the bladder by cystoscopic examination. Total cystectomy with construction of ileal conduit was performed. Histologically, the tumor was composed of carcinomatous and sarcomatous elements. The carcinomatous element was compatible with grade 3 transitional cell carcinoma. The sarcomatous element was composed of osteosarcomatous, chondrosarcomatous pattern and undifferentiated malignant spindle cell component. Immunohistochemical examination demonstrated the presence of keratin, cytokeratin and epithelial membrane antigen in both carcinomatous and sarcomatous elements. Therefore, we diagnosed this tumor as sarcomatoid carcinoma. The patient has remained well without any evidence of recurrence for 10 months after operation.

A 67-year-old man visited our hospital with the chief complaint of painless swelling of the left scrotal content. An elastic hard mass was palpable in the left scrotum. Resection of the tumor, including the left scrotal skin, was performed. The tumor originated from the scrotal wall and did not communicate with the testis, the epididymis or spermatic cord. Histologically, the tumor was diagnosed as a malignant mesenchymoma composed of liposarcoma, chondrosacoma and osteosarcoma. Malignant mesenchymoma is very rare, especially in the scrotum. Our case that originated from the scrotal wall is the first one reported in Japan.

A 71-year-old man was admitted our hospital because of mediastinal tumor. On chest X-ray, the mass was about 10 cm in diameter, contact with the right side of No. 3-5 vertebra and extended to the right thorax. After about 8 months, he felt anterior chest and back pain. Tumor was growing on chest X-ray and, on chest CT, linear and spotty calcification existed in it. On MRI, T1 weighted image, most part of tumor was low signal area but high area was also existed and it was parenchymatous hemorrhage. On T2 weighted image, tumor was high signal and irregular low area was calcification. Operation was performed and a hard tumor, measured 12 x 9 x 6 cm, originated from the 3rd-4th rib was resected. Histopathological diagnosis was chondrosarcoma. MRI was effective for the diagnosis of this case.

We report a case of intracranial chondrosarcoma of the skull base with fatal intra- and peritumoral hemorrhage. A 75-year-old woman complained of right blepharoptosis and diplopia in 1989. An initial diagnosis of Tolosa-Hunt syndrome was made, and the patient was treated with steroid hormone therapy at a local hospital. Because the symptoms had not been relieved, she was admitted to our hospital. Computed tomography (CT) scan and magnetic resonance (MR) images demonstrated a large mass extending from the right side of the clivus to the parasellar region and petrous apex. The mass was partially calcified and had destroyed the base of the middle cranial fossa. The lesion had homogeneous enhancement with contrast medium. Preoperative diagnosis was chordoma or chondroma. A biopsy of the tumor was made. The pathological diagnosis of biopsy specimen was chondroid chordoma. The patient was followed up but no palliative treatment such as radiotherapy was given. On June 25, 1991, she suffered from cerebral infarction. On June 29, 1993, she died of sudden respiratory failure. Autopsy was performed. It revealed intra- and peritumoral hematoma compressing the medulla oblongata, pons and midbrain. Histologically immature chondroid cells proliferated in a myxoid-rich extracellular matrix. The tumor cells were composed of hyperchromatic nuclei and eosinophilic cytoplasm, but there was no evidence of notochordal differentiation. Compared with biopsy findings, the tumor showed high cellular density. Immunohistochemically, the tumor cells reacted positively for S - 100 protein, vimentin and cytokeratin, but negatively for epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA). In view of these histopathological findings, the diagnosis of low-grade myxoid chondrosarcoma was established. Intratumoral hemorrhage often occurs in malignant brain tumors such as glioblastoma and metastatic brain tumor, but chondroid tumors rarely develop a fatal type of intratumoral hemorrhage. Only 8 cases have been reported in detail to date. We discuss the immunohistochemical features and spontaneous intratumoral hemorrhage of chondrosarcoma.

A 17-year-old female, who had diplopia and progressive gait disturbance, had been diagnosed as having a right parasellar mass lesion in 1986. Initial CT scan failed to show a parasellar space occupying lesion, although it was well demonstrated on MRI. No surgery was carried out at that time. Seven years later, she was hospitalized because of progressive gait disturbance, lower cranial nerves palsy, and cerebellar sign as well as the known right abducence palsy. At this time, CT scan exhibited a huge low density area with a marginal high density area suggesting calcification, which extended from the right parasellar area to the ventral portion of the midbrain. She underwent surgery under the diagnosis of cartilaginous tumor or chordoma. The intracranial lesion was removed via combined translabyrinthine approach and epidural subtemporal approach. The tumor was hitologically diagnosed as well-differentiated chondrosarcoma. Electron-microscopy demonstrated tumor cells with features of chondrocytes. Since the original site of the tumor, strategies for treatment and prognosis are different from each other, distinction between chondrosarcoma and chordoma is important. MRI seemed very useful for making this distinction as a first step.

Chondrosarcoma of rib origin is rare in Japan. A 51-year-old man came to our hospital with chest pain and coin lesion in the left superior part of the chest X-ray film. It was suspected to be the posterior mediastinal tumor based on CT and MRI. Operation was done to remove the tumor and the histological diagnosis was chondrosarcoma. The tumor measured 2.5 x 2.6 x 2.2 cm arising from the posterior portion of the left third rib. Combined resection of descending aortic adventitia was done. The patient is doing well without symptoms of recurrence 15 months after operation.

Mesenchymal chondrosarcoma is a very rare occurrence in the orbit. A 10-year-old girl presented with right exophthalmos. Computed tomography revealed a round tumor inside the muscular cone of the right orbit. There was a sign of slight calcification inside the tumor. The tumor was surgically removed by the Krönlein-Berke procedure. Histopathological examination showed undifferentiated mesenchymal cells and cartilage tissue. A recurrent tumor was found in the same location 34 months after the surgery, which was removed by frontal approach saving the globe with normal function. Histopathology of the recurrent tumor revealed a slight difference from the primary tumor, showing hemangiopericytoma pattern without cartilage components. Immunohistochemical studies of the primary and recurrent tumors showed their cells to be positive for antifactor VIII and S-100, and negative for myoglobin and monoclonal antibody to muscle actin. Electron microscopically these undifferentiated cells had large nucleus and very scanty cytoplasm, mostly containing glycogen granules. This is probably the first description of a juvenile case of mesenchymal chondrosarcoma originating from soft tissue in the orbit.

A case of 70 years old female bearing a primary cardiac tumor was presented. Mitral valve replacement and reconstruction of the interatrial septum were necessary because the tumor originated from the left atrial side of the interatrial septum and invaded the anterior mitral valve leaflet and around the annular area. Pathological examination of the resected specimens revealed mesenchymal tumor containing chondrosarcomatous component. The patient received radiation therapy (50 Gy) postoperatively and she resumed normal daily activity. Preoperative echocardiographic manifestations were of proved benefit in the diagnosis of malignant cardiac tumor and a proper therapeutic strategy could be chosen.

The case of a 48-year-old male with a malignant mesenchymoma of the maxilla is reported. The patient had complained of bloody rhinorrhea and cheek pain, and a total maxillectomy was performed. The tumor consisted of a rhabdomyosarcoma, an osteosarcoma and a chondrosarcoma, which were relatively differentiated. Later, recurrence of a tumor and a lung metastasis occurred, however the tumor revealed undifferentiated mesenchymal cells. This led us to suspect the origin of the malignant mesenchymoma had been a primitive mesenchymal cell. To our a knowledge, the present case is the first reported case of a malignant mesenchymoma with a maxillary origin in the world.

A 52-year-old woman admitted with difficulty of breathing and had an anterior chest wall tumor. Primary lesion of the chest wall tumor invading the inferior one third of sternum, right third to sixth ribs, a part of the right middle lobe, the pericardium and a part of the diaphragma and small nodules on the parietal pleura were resected, and a diagnosis of chondrosarcoma of the right fifth or sixth rib and pleural dissemination of the tumor was established. The defect of pericardium was repaired with artificial dura mater and the defect of bony chest wall was repaired with a double layer of Marlex mesh. Although the resection was palliative because of pleural dissemination, she is now working as a housewife without difficulty of breathing.

During the 6-year period of 1976 to 1981, we studied 6 cases of mixed mesodermal tumor of the uterus. The incidence of occurrence of the tumor was 0.4% of all the gynecological inpatients, 1.4% of all the uterine malignancies, and 9.5% of the uterine body malignancies, and was higher than those reported by others. The patients were aged 49-75 years (mean 64 years), and 5 out of 6 patients were postmenopausal. Four patients had heterogeneous tumors (3 rhabdomyosarcomas and 1 chondrosarcoma). Two of the 6 patients are now living 34 and 14 months after treatment without clinical evidence of recurrence, respectively. Histologically, one of them showed only benign glandular structures as a component of epithelial elements, and the other one had no epithelial element. These findings suggest that the malignancy of the epithelial components may be one of the most important prognostic factors in the mixed mesodermal tumor of the uterus.

The patient was a 52-year-old woman who was initially admitted to our hospital with a recent history of diplopia, ptosis and visual impairment on the right, adding to 6 years history of the right orbital pain and temporal headache. She had gradually developed pain and fatigue of the right knee since childhood and had been suffered from multiple subcutaneous tumor in the both hands and the right foot from youth onward. On neurological examination, she has had right exphthalmos and 3rd and 6th cranial nerve palsies on the right. Skull X-ray and CT scan showed parasellar calcified mass on the right. In chromosome examination, the inversion of the No. 1 chromosome was disclosed by the Q- and C-band dyeing of her leucocytes and skin culture. Cavernous hemangioma of her skin lesion and enchondroma of the skeletal lesion were diagnosed by X-ray examination and histological specimen, indicating so-called Maffucci's syndrome. One year and a half later, she was readmitted to our institution, because of progressive visual loss on the right and left hemiparesis. Malignant change of the parasellar lesion was suspected by the clinical course and neuroradiological findings and was confirmed histologically by the second peration. During postoperative course, intratumoral hemorrhage had occurred twice and the patient died, and postmortem examination confirmed her clinical affair. This is the case which was clarified malignant transformation of enchondroma in the parasellar region, and is the first case verified the chromosome abnormalities in Maffucci's syndrome.

Two cases were reported in which malignant cardiac tumors attached to the mitral valve were diagnosed during their lifetimes using two-dimensional echocardiography. Case 1 was a 29-year-old man with speech disturbance and left hemiparesis. Histological examination of the specimen excised from the cardiac tumor during the operation revealed mesenchymal chondrosarcoma, which is extremely rare etiologically and has never been reported so far. Case 2 was a 62-year-old woman complaining of paroxysmal nocturnal dyspnea and chest pain. The diagnosis of malignant histiocytosis was made from the pathological examination of biopsy specimen taken from the rib metastasis. Using two-dimensional echocardiography, characteristic findings for the cardiac tumor were obtained. The tumor echo in Case 1 showed, unlike to that reported for myxoma, two different echogenic layers; the outer dense and the inner light in the echo density. On surgery, tumor echo was revealed to reflect the cystic lesion. In Case 2, two-dimensional echocardiography on admission revealed two separate tumor echoes which attached to the mitral valve and left atrial wall region, respectively. Within two months, they grew rapidly and finally fused into one mass resulting in so-called ball valve syndrome. Phonocardiographically, the tumor plop in Case 1 was high-pitched in quality, and was extinguished completely after the tumor was resected. The tumor plop in Case 2 was not audible on admission, but became evident after fusion of the tumor echoes and was associated with a presystolic murmur.

A case of a breast tumor which showed the appearance of invasive ductal carcinoma and chondrosarcoma is reported. The patient was a 36-year-old female. Simple mastectomy and lymph node dissection were performed for the tumor. Local recurrence and metastases to the contralateral breast, lymph nodes, subcutaneous tissue, and bones arose after the operation. She died of the disease 2 years and 3 months after the operation. It is thought that this tumor had formed as a result of metaplasia of the carcinomatous tissue, since a histological transition between the carcinoma and sarcoma was demonstrated.

A case of so-called gastric carcinosaroma in a 70-year-old man was presented. The primary tumor measuring 5.6 X 5.4 cm was located in the gastric antrum and had the appearance of Borrmann 2 type. A massive metastatic tumor was found in the pyloric lymph nodes. Histologically, the primary tumor was predominantly composed of papillary adenocarcinoma invading to the muscle layer with lymphatic and vascular involvements. Atypical spindle cells occasionally intervened between carcinoma nests. The metastatic tumor consisted of mixed papillary adenocarcinoma and well differentiated chondrosarcoma with transitional areas by atypical spindle cells. Therefore, it was suggested that sarcomatous components in the present case were derived from carcinoma cells by undifferentiated. Three cases of gastric carcinosarcoma reported in Japan were reviewed, and their histogenesis was discussed as compared with the present case.

A 40 year-old female underwent mastectomy for a rapidly growing left breast mass. Histologically, the tumor was composed of invasive ductal carcinoma with squamous metaplasia, a cartilaginous component and a pleomorphic sarcoma-like component. In each histologic feature, gradual transition was recognized. A diagnosis of carcinoma with metaplasia (squamous and cartilaginous type) of the breast was made. The cartilaginous component in this case is probably the result of direct cartilaginous metaplasia of carcinoma cells and cartilaginous metaplasia followed upon carcinoma cells into small undifferentiated tumor cells and pleomorphic sarcomatous cells.

A rare case of mesenchymal chondrosarcoma originating from the thoracic spinal dura was reported. A 10-year-old girl complained of paresis of the right leg in June 1977. Then she became unable to walk early in August, and a complete paraparesis developed on August 18. She was admitted to our department on August 22. On admission she had complete paraparesis, bilateral ankle clonus, upward plantar reflexes, sensory disturbance below T7, shincter disturbance and neck stiffness. Plain thoracic X-ray revealed bilateral decalcification of pedicles of T6. Myodil myelography showed a complete block between T6 and lower end of T7 vertebrae. Bilateral laminectomy from T3 to T8 was performed. A tumor originating from the spinal dura was located in the right dorsal extradural space. The tumor was totally removed together with a small area of the affected dura. Light microscopy showed mesenchymal chondrosarcoma. Her recovery from neurological deficiencies was excellent and now she can run 14 months after surgery. Metastasis or recurrence has not yet been seen.

Internal pressure and oxygen tension were measured in 24 patients with bone tumors and tumorous conditions. High internal pressure were observed in most of the rapidly growing lesions. The internal pressure of slowly growing and non-growing lesions were not significantly different from those of normal bone marrow. Oxygen tension was commonly higher in the rapidly growing lesions than in simultaneously obtained peripheral venous samples from the same patients. A high correlation was found between internal pressure and the growth rate of the bone lesions in this study. These data suggest that internal pressure and oxygen tension of the lesions reflect the degree of blood supply to the bone tumors and tumorous conditions.