Synovial sarcomas usually metastasize after >5 years: a multicenter retrospective analysis with minimum follow-up of 10 years for survivors.

BACKGROUND: Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years that evaluate long-term outcomes for survivors. PATIENTS AND METHODS: Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. Mean follow-up of living patients was 17.2 years and of dead patients 7.7 years. RESULTS: Mean age at diagnosis was 35.4 years (range 6-82 years). Overall survival was 38.7%. The 5-year survival was 74.2%; 10-year survival was 61.2%; and 15-year survival was 46.5%. Fifteen patients (24%) died of disease after 10 years of follow-up. Local recurrence occurred after a mean of 3.6 years (range 0.5-14.9 years) and metastases at a mean of 5.7 years (range 0.5-16.3 years). Only four patients were treated technically correctly with a planned biopsy followed by a wide resection or amputation. Factors associated with significantly worse prognosis included larger tumor size, metastases at the time of diagnosis, high-grade histology, trunk-related disease, and lack of wide resection as primary surgical treatment. CONCLUSIONS: In SS, metastases develop late with high mortality. Patients with SS should be followed for >10 years.

Intraosseous myelolipoma.

Two patients with lesions in the acetabulum and femur, evident on imaging, were found to have normal marrow elements without a histopathological lesion on curettage of the acetabulum and resection of the femur, respectively. Radiographs on the first patient showed a sclerotic benign appearing acetabular lesion reminiscent of a fibro-osseous lesion while MRI showed the entire lesion to contain fat. The histological features were neither of a fibro-osseous lesion nor a lipoma, but only normal to mildly hypercellular marrow elements. The second patient had a long and expansive femoral osteolytic lesion which on biopsy showed cellular marrow. A subsequent stress fracture led to resection and prosthetic replacement. Microscopic findings revealed only cellular hematopoietic marrow with mature red and white cell lines, megakaryocytes, and a few mast cells devoid of trabecular bones. The microscopic features in the first patient raised the possibility of hematopoietic hyperplasia. Neither the radiographic nor MRI features were consistent with that diagnosis. The spectrum of imaging findings and microscopic appearances in both patients best fit the diagnosis of intraosseous myelolipoma.

Late solitary bone metastasis of a primary pulmonary synovial sarcoma with SYT-SSX1 translocation type: case report with a long follow-up.

Primary synovial sarcoma outside its classical presentation in para-articular soft tissue of young patients is rare but regularly reported. One of the rarest primary locations is the lung. We describe a 73-year-old female patient who presented with a solitary malignant bone tumor 8 years after the resection of a lung neoplasm. The bone tumor was classified as an osteosarcoma and the lung tumor as an atypical carcinoid tumor at their first respective diagnostic work-ups. The resection of the affected humerus with allograft and endoprosthesis implantation followed. Reevaluation of the tumor samples at the time of the local recurrence of the bone tumor 6 years following the initial symptoms of the bone tumor lead to the reclassification of both specimens as synovial sarcomas. Both neoplasms contained the SYT-SSX1 type of the diagnostic translocation t(X;18) as detected by the reverse-transcription polymerase chain reaction analysis. The patient died 14 years after the resection of the primary synovial sarcoma of the lung and 6 years following the occurrence of the bone metastasis. This prolonged clinical course is uncommon for the SYT-SSX1 translocation, which, in other locations, is usually associated with an unfavorable prognosis.

[Giant cell tumor of bone with rapid malignant course]. / Riesenzelltumor des Knochens mit rapid malignem Verlauf.

The case of a 28-year-old male patient with a locally aggressive lesion of the distal tibia is presented. Following the diagnosis of giant cell tumor of bone (GCT) on biopsy and curettage, a rapid malignant course was observed with recurrence 2.5 months later. Multiple metastases appeared 6 months after initial presentation. Following initial chemotherapy according to the COSS protocol and later with carboplatin and VP-16, therapy was changed to Adriamycin and later gemcitabine due to progressive disease. Good palliation was achieved, and the patient felt well with less shortness of breath on exertion and was ambulatory with walking aids. The malignant nature of the tumor was not detected in the initial pathologic examinations. Review of the pathologic material provided histologic clues permitting the diagnosis of a primary malignant GCT with a fibrohistiocytic/fibrosarcomatous component. Malignancy in a giant cell tumor is a much debated diagnostic dilemma when a frank sarcomatous component is lacking. Cytologic atypias and flame-like tufts of infiltration of soft tissue are important clues. Surgical treatment should be commensurate. Monotherapy with Adriamycin or gemcitabine can be considered in order to inhibit the disease progression.

Gorham-Stout disease of the shoulder girdle and cervico-thoracic spine: fatal course in a 65-year-old woman.

A case of Gorham-Stout disease of the shoulder girdle and cervico-thoracic spine in a 65-year-old woman is described. The patient presented with progressive neurologic symptoms, pain, and deformities of the cervico-thoracic spine as well as of her left shoulder following a traumatic shoulder luxation. Since the patient had a history of uterine carcinoma, the current disease was clinically difficult to differentiate from osteolytic metastases. The results of the clinical, radiological and histopathologic examination leading to the diagnosis of Gorham-Stout disease are described. Neither attempted surgery nor radiotherapy produced clinical improvement and the patient died 1 year after the first clinical evaluation.

Osseous lesions of the distal ulna: atypical location--unusual diagnosis. Report of three cases with similar imaging and different pathologic diagnoses.

Three cases with destructive bone lesions of the distal end of the ulna caused by different pathologic entities (Ewing's sarcoma, osteosarcoma, rheumatoid pseudotumoral synovitis) are presented, all with similar clinical and comparable x-ray and magnetic resonance imaging features. Although the distal end of the ulna may be resected without significant functional impairment, careful evaluation of treatment strategies compatible with oncologic standards is warranted.

[Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation and glandular component]. / Tumeur maligne de la gaine des nerfs périphériques à différenciation rhabdomyoblastique et à composante glandulaire.

An exceptional case of malignant peripheral nerve sheath tumor with striated muscle differentiation and glandular component is reported, in a 52-year old man. This tumor measured 8 cm in diameter, and was localized in the chest wall, infiltrating the skeletal muscle. The mesenchymal portion of the tumor was composed mostly of spindle cells arranged in interlacing fascicles. Between these fascicles, there were large cells with abundant eosinophilic cytoplasm and clear elongated nucleus. Immunohistochemical study demonstrated cytokeratin, EMA and CEA expression in the glandular component and S100 protein expression in the major portion of the mesenchymal component. The large cells identified as rhabdomyoblasts, expressed desmin, myoglobin, alpha-SR actin and alpha-SM actin. The tumor recurred 5 years after its resection. Histological and immunohistochemical features were identical. We think that positivity of neoplastic striated muscle cells with alpha-SM actin reflects an early differentiation phase of these cells.

[Neoadjuvant therapy for localized osteosarcoma of extremities. Results from the Cooperative osteosarcoma study group COSS of 925 patients]. / Neoadjuvante Therapie des lokalisierten Osteosarkoms der Extremitäten. Erfahrungen der Cooperativen Osteosarkomstudiengruppe COSS an 925 Patienten.

BACKGROUND: Owing to twenty years of multicentric interdisciplinary cooperation, the COSS group has been able to collect data on a large group of osteosarcoma patients treated by neoadjuvant therapy. This paper reviews results achieved in patients with localized extremity tumors. INCLUSION CRITERIA: Registration into a completed neoadjuvant COSS-Study. Histologically confirmed, primary, localized, high-grade, central osteosarcoma of an extremity; age < 40 years; no pretreatment; interval diagnosis to chemotherapy < or = 3 weeks; no severe comorbidity. Chemotherapy: HD-methotrexate +/- doxorubicin +/- cisplatin +/- ifosfamide +/- BCD. Scheduled local therapy: Surgery. RESULTS: 925 evaluable patients from 101 institutions. Median age 15 years, m:f 1.4:1. Primary site: femur 510, tibia 251, humerus 100, fibula 51, other 13. Tumor-size < 1/3 of the involved bone 616, > or = 1/3 304. Definitive surgery in 903/925 cases, 443 limb salvage procedures. Good response (> 90% necrosis) in 469/806 (58.2%) evaluated tumors. Median follow-up for surviving patients: 5.42 years. Actuarial survival after 5 and 10 years: 72.5% (95%-CI 69.3-75.7) and 66.3% (62.5-70.0), relapse-free 62.1% (58.7-65.4) and 59.4% (55.8-63.0). 683/925 alive (601 first remission), 242 deceased (212 tumor progression, 30 other causes). 66.2% (97.3%) of all relapses within 2 (5) years. Prognosis correlates with tumor-size (< vs. > or = 1/3: 69.9% vs. 58.3% at 10 years) and -site (tibia: 74.2%, humerus: 54.5%) and -response (good vs. poor: 78.2% vs. 52.5%) (all p < 0.01). Actuarial 10-year survival by response grading I-VI according to Salzer-Kuntschik 80.9%, 82.8%, 71.1%, 60.7%, 47.7%, 27.3%. COSS-studies with preoperative 4-drug therapy more efficacious than less aggressive protocols. No impact of doxorubicin scheduling (sequential: rapid vs. 48 h-continuous infusion) or cisplatin scheduling (randomized: 5 h vs. 72 h-infusion) on prognosis detected. CONCLUSIONS: Intensive multiagent chemotherapy and delayed surgery for localized extremity osteosarcoma led to excellent oncologic results in the COSS-studies. Tumor-size, -site, and -response as well as the intensity of upfront chemotherapy correlated with outcome. Giving doxorubicin and cisplatin by continuous infusions did not result in discernible prognostic disadvantages.

Chondromyxoid fibroma of two thoracic vertebrae.

We report on a case of chondromyxoid fibroma involving two adjacent thoracic vertebrae with features of aggressive behaviour on radiographs, CT and MRI. Histology revealed typical chondromyxoid fibroma with unusually coarse calcifications. Chondromyxoid fibroma of the spine is rare, and only 30 of these tumours have been reported so far. Involvement of two contiguous vertebral bodies by chondromyxoid fibroma, as reported here, appears exceptional.

Analysis of two bone-prosthesis interfaces and membranes from non-cemented trapeziometacarpal prostheses.

The non-cemented Ledoux trapeziometacarpal prosthesis has a high failure rate. In order to better understand the mechanism responsible for pain and loosening, we thoroughly analysed the membranes surrounding the prosthesis and the bone-prosthesis interface in two trapezia, each containing a Ledoux cup, which were resected after unsuccessful implantation. Serial sections, perpendicular to the longitudinal axis of the implanted cup, allowed histological examination of the interface and were used to quantify bone apposition. The tissues surrounding the prosthesis showed a foreign-body reaction to particles identified as titanium. The interface showed bony integration of the cup, mainly on the radial side and on the proximal part of the cup, with an appositional index of 28%. Our findings suggest that bony apposition might not be sufficient to ensure successful anchoring of the Ledoux cup in the trapezium in the presence of an intense foreign body reaction to titanium. Moreover, the presence of metal might be secondary to micromotion of the wings of the metallic part of the cup, induced by axial movement of the underlying polyethylene during pinch grip. The combination of polyethylene as an expander and titanium may need to be reviewed.

[Muscle metastasis as initial manifestation of adenocarcinoma of the stomach]. / Muskel-Metastase als Erstmanifestation eines Adenokarzinoms des Magens.

BACKGROUND: The presentation of gastric carcinoma with a metastasis in skeletal muscle is a distinct rarity. CASE REPORT: In a 72-year-old male patient a soft tissue mass behind the knee was biopsied rather late. Histology showed a metastasis of adenocarcinoma. The patient mentioned a slight feeling of strangulation, which led to endoscopy and the diagnosis of gastric carcinoma. Palliative chemotherapy was initially successful but the patient died after 21 months. DISCUSSION: Based on this case history, three points are discussed: how to biopsy a peripheral soft tissue mass, how to proceed when a metastasis of an unknown primary tumor is diagnosed, and the epidemiology of metastases to skeletal muscle.

[Magnetic resonance tomography in osteoid osteoma: more confusion than benefit?]. / Magnetresonanztomographie beim Osteoid-Osteom: mehr Verwirrung als Nutzen?

The purpose of this evaluation was the description of potentially misleading MR appearances of osteoid osteoma. The MR images of 10 patients with osteoid osteoma were retrospectively evaluated and compared to radiographic, intraoperative and histologic findings. 4 of the 10 abnormalities were located in the proximal femur, two in the lumbar spine, and one each in the tibial plateau, in the cervical spine, in the sacrum and in the first metacarpal. 8 of the 9 nidi visible on standard radiographs and/or CT scans were demonstrated on MR images. Edema was visible within the bone marrow in 3, within soft tissue in 2 and in both locations in 4 MR examinations. The soft tissue abnormalities were circumscribed in 3 patients and could be misdiagnosed as soft tissue tumors or an abscess in these patients. One of 5 osteoid osteomas located in the proximity of a joint mimicked septic arthritis. The MR appearance of osteoid osteoma may be misleading. However, false diagnoses usually can be avoided with a careful search of a nidus. MR imaging in osteoid osteoma is important for differential diagnosis.

Langerhans'-cell histiocytosis in adults.

Guided by a long-term retrospective observation, the clinical course and treatment of Langerhans'-cell histiocytosis (LCH) in adult patients are represented. The series included 19 patients meeting the histopathologic criteria of presumptive LCH who were followed for 1.5-20 years (average 7.7 years). Most frequently, skeletal lesions (16 patients), diffuse interstitial lung infiltrates (seven patients), and pituitary gland involvement with diabetes insipidus (four patients) were present. Bone lesions of the skull and axial skeleton were associated with an infiltration of adjacent soft tissues in 10 of 16 patients. Liver, lymph node, and bone marrow involvement appeared sporadically. LCH was divided into localized or multifocal form. Localized disease took a benign course with remission of bone (n = 4) or lymph node lesions (n = 2). Also, in isolated pulmonary LCH (n = 2), spontaneous transition to inactive disease occurred. With the exception of isolated bone lesions (n = 27), which remained asymptomatic or showed a remission to treatment, multifocal LCH had a more aggressive course. Osseous lesions with adjacent soft tissue infiltration (n = 20) showed a relapse rate in excess of 80% independent of the treatment applied. Pulmonary involvement led to a more marked functional impairment compared to the isolated form, and systemic treatment yielded no convincing effect. In three patients with liver or bone marrow involvement, LCH showed a persistent, serious disease activity. One patient died of transition into acute monomyelocytic leukemia 18 months after diagnosis without preceding chemotherapy. In adults, LCH seems to be limited to a few organ systems. Multifocal LCH represents the more aggressive form with unfavorable prognosis in patients with bone lesions spreading into the adjacent soft tissue and liver or bone marrow involvement.

Imaging the long head of biceps tendon--a pictorial essay emphasizing magnetic resonance.

Lesions of the long head of the bicepstendon (BT) are seen in association with tears of the rotator-cuff, particularly lesions of the subscapularis tendon and the rotator-interval. The frequency of positive MR-findings at the BT is approximately 25%. The pathologic alterations include complete medial luxation, subluxation and entrapment by the subscapularis tendon, tendinitis or tendovaginitis and lesions at the origin of the tendon at the superior labrum (superior labrum anterior to posterior (SLAP)--lesions). The imaging signs of BT pathology on MR include an abnormal course and position of the tendon, alterations in shape and changes in signal-intensity (SI), obliteration and thickening of the tendon-sheath. In long standing intra-articular BT rupture, neo-insertion of the tendon in the bicipital sulcus may ensue. The long BT needs to be visualized in transaxial, coronal oblique and sagittal oblique projections. Besides serving as stabilizer of the long head of biceps muscle, the BT is also an important stabilizer of the anterior joint capsule.

[Osteosarcoma and Ewing's sarcoma. Improved recovery chances in the past 25 years according to the experience of Balgrist Hospital]. / Osteosarkom und Ewing-Sarkom. Die verbesserten Heilungschancen in den vergangenen 25 Jahren in der Erfahrung der Klinik Balgrist.

At the Balgrist hospital 38 patients with osteosarcoma and 18 patients with Ewing sarcoma were treated from 1970 through 1992; a follow-up of at least three years is available for all patients. Disease-free survival in this period of time has increased from 20% (Ewing sarcoma) and 30% (osteosarcoma) to over 75%. While systemic tumor control was improved by systematic chemotherapy, local tumor control was improved by appropriate biopsy techniques, and amputations have been replaced by local resections, in most cases with reconstruction (arthrodesis, rotation plasty, endoprosthesis, allografts), which require further improvements. With respect to cure of the neoplastic disease, the currently available methods have been used to their maximum potential, and a steady state has been reached regarding survival. Further improvement of survival will therefore depend on the introduction of completely new modalities.

Hyperostotic and destructive osteoarthritis in a patient with vitamin A intoxication syndrome: a case report.

The authors present a case report of a 59-year-old female suffering from hyperlipidemia who developed chronic vitamin A intoxication syndrome after ingestion of 30,000 IU retinol/daily over a period of six years. The patient's main complaints included severe headaches, morning nausea, myalgias and disability around the hip, knee, and ankle joints. Radiologically, hyperostosis of the acetabular circumference and the spine was demonstrated. Because of rapidly increasing pain, total hip replacement was performed. Histology of cross sections from the femoral head revealed destructive osteoarthritis. Since no other causative reason was found, retinol may not only be responsible for hyperostotic bone and soft tissue formations but may perhaps also account for rapid progressing of degenerative joint disease. Despite the cessation of vitamin A intake the clinical symptoms persisted due to hyperlipidemia. The enlarged number of chylomicrons and the higher fraction of very low density lipoproteins may represent a second retinyl ester pool in case of overloaded fat storing Ito-cells in the liver. Therefore, rheumatological treatment reducing risk factors such as hyperlipidemia is mandatory.

[Stewart-Treves syndrome after breast carcinoma]. / Stewart-Treves-Syndrom nach Mammakarzinom.

The Stewart-Treves syndrome is an angiosarcoma that occurs due to chronic lymphoedema which in most cases is a complication after mastectomy with axillary node dissection and postoperative radiation. Prognosis for this rare tumour is poor. The best therapy is early and radical excision. Chronic lymphoedema seems to be an important pathogenetic factor. We report on a 70-year old patient with early stage lymphangiosarcoma who had a lumpectomy with axillary node dissection and postoperative radiation five years ago.

Optimization of prostate carcinoma staging: comparison of imaging and clinical methods.

PURPOSE: The diagnostic value of endorectal coil MRI, body coil MRI, transrectal ultrasound, digital rectal examination and PSA levels were prospectively analysed in order to define the most accurate preoperative staging method. METHODS: 33 patients with prostate carcinoma, who underwent subsequent prostatectomy, were enrolled in the study and examined on a 1.5T system using the body coil as well as the endorectal surface coil before and after the administration of contrast material. The results were compared to digital rectal examination, prostate specific antigen levels and endorectal ultrasound. RESULTS: Staging accuracy of endorectal coil MRI was 87.9% with a sensitivity of 88.9% and specificity of 86.7%. For body coil MRI, the staging accuracy was 75.7%, the sensitivity 66.7% and the specificity 87.9%, for transrectal ultrasound 69.6%, 41.7% and 100% and for the digital rectal examination 56.6%, 33.3% and 100%, respectively. Prediction was improved by combining results of endorectal coil MRI with PSA values. CONCLUSION: Endorectal ultrasound and digital rectal examination both had a tendency to underestimate the extent of the lesion. Endorectal coil MRI proved to be the best preoperative staging method. In combination with PSA values, diagnostic accuracy could be further improved. Therefore, local staging of prostate cancer could be based on these two parameters alone.

Loose bodies in the temporomandibular joint. The advantages of arthroscopy.

Loose bodies are a rare cause of temporomandibular joint symptoms. Their main source is synovial chondromatosis. We report on clinical findings, diagnostic methods, treatment choices and outcome following the removal of loose bodies in 10 patients. Seven patients were evaluated and treated by means of arthroscopy, while in three patients open arthrotomy was performed. In five patients, no diagnostic imaging technique had demonstrated the presence of loose bodies prior to arthroscopy. In six patients, histology revealed synovial chondromatosis. In four patients, osteochondral fragments alone were found. Until now, the recommended treatment of choice for the removal of all loose bodies and of affected synovial tissue required open arthrotomy. We conclude that the advantages of arthroscopy consist in locating loose bodies that are not detectable radiologically and in reducing operative trauma.