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Biomedical subjects

A von Hochstetter

Publications and source records attributed to A von Hochstetter.

At least 19 recordsLinked to original sources

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.

Aged↗

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.

Aged↗

[Magnetic resonance tomography in osteoid osteoma: more confusion than benefit?].

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.

Adolescent↗

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.

Adult↗

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.

Humans↗

[Stewart-Treves syndrome after breast carcinoma].

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.

Aged↗

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.

Adolescent↗

Diagnostic accuracy of TMJ arthroscopy in correlation to histological findings.

From 1987-1993, 356 arthroscopic examinations of the temporomandibular joint were performed on 295 patients. During 69 examinations, biopsies were obtained to correlate arthroscopic findings with histology. In the overall group, histology confirmed in 78.9% the arthroscopic findings. Correlation was better in joints with degenerative changes (81.5%) than in those with a synovitic/hyperaemic appearance (61.5%). Chondroid metaplasia, detritus synovitis and synovial chondromatosis were additional diagnoses given by histological examination. In 11 joints, open arthrotomy was performed after arthroscopy with biopsy was carried out. The excised tissue was also investigated microscopically and correlates to the biopsy-result. Because no different pathological changes were found, it is concluded that biopsies performed during arthroscopy of the temporomandibular joint are representative for histological investigation. An additional perforation for introducing the biopsy forceps is not necessary, because results obtained with different techniques appeared to be equally accurate.

Adolescent↗

Valgus tibial osteotomy in a patient with benign dominant osteopetrosis (Albers-Schoenberg disease). A case report.

Operation in osteopetrosis, or Albers-Schoenberg disease, needs careful technique because of the brittleness of the bone. We report a patient with autosomal-dominant osteopetrosis (MIM 166600)18 and unicompartmental osteoarthritis of the knee which was treated by a valgus tibial osteotomy. Our experience, and a review of the literature, shows that external fixation is a valuable form of treatment because it is noninvasive and ensures perfect interfragmentary compression.

Aged↗

[Cytokeratin-positive angiosarcoma of the adrenal gland].

Cytological, biopsy and autopsy findings in a patients suffering from massively metastasizing adrenal angiosarcoma are reported. Histogenetic typing of the tumour initially manifestating itself by osseous and liver metastases was problematic with regard to its partially epithelioid structure and its positivity upon cytokeratin immunostaining. Of relevance for the correct typing was the finding that the tumour cells in addition exhibited positivity for vascular markers. This case confirms literature data according to which cytokeratin expression not infrequently may be encountered in endothelial neoplasms and which by no means should lead to exclude such a tentative diagnosis.

Adrenal Gland Neoplasms↗

[Combination of surgical therapy and interventional radiology in a recurrent myxoid liposarcoma with a 37-year history].

Extreme long-term survivals of low grade liposarcomas are rare. The authors present a case of a 75 year old man with a 37 year history of recurrent myxoid liposarcoma. The tumor dynamics are obviously related to the histology subtype. The clinical and radiological findings of the sixth tumor recurrence are discussed. The combination therapy of surgical tumor reduction and interventional radiology with implantation of endovascular prostheses for iliaco-femoral vein compression due to tumor recurrence is described.

Abdominal Neoplasms↗

Zürich experience with preoperative, high dose methotrexate-containing chemotherapy in patients with extremity osteosarcomas (OSA).

From 1979 to 1990, 37 patients with extremity osteosarcomas, 22 of them males and 15 females, median age 19 years, received pre- and postoperative chemotherapy. The period of observation, calculated from after the primary operation, ranged from 1-114 months, median 25 months. After preoperative chemotherapy, 9 (24%) underwent a primary amputation, in 28 (76%) a limb salvage procedure was possible, 4/29 (14%) later developed local recurrences, metastases were diagnosed in 8/9 amputees and 5/28 after limb-sparing surgery. The five-year disease-free and overall survivals after amputation are 11% and 33%, respectively, compared to 68% and 75%, respectively (p = 0.001 and 0.018, respectively, for long rank). Results of histologic assessment after preoperative chemotherapy are of significant prognostic impact. The earlier prognostic groups of 0%-49% necrosis versus 50%-100% necrosis were statistically no longer suitable for distinguishing useful prognostic groups. In this second analysis, patients with 0%-79% necrosis versus 80%-100% necrosis had 5-year disease-free survivals of 81% versus 44% (p = 0.032) and 5-year overall survivals of 88% In summary, 24% of our patients with extremity osteosarcomas, most of them with large primaries close to joints, had to undergo primary amputation and had only a 33% 5-yr-survival, whereas limb salvage procedures with pre-and postoperative chemotherapy were associated with a 5-yr survival of 75% and thus had no adverse impact. Careful selection of patients for successful management of osteosarcomas is important; necrosis of 80% and more after preoperative chemotherapy is a prerequisite for a favourable outcome.

Adolescent↗

[Eosinophilic fasciitis (Shulman's syndrome)].

A 36-year-old man had noted a firm and painful swelling of the upper and lower limb, increasing over the previous few weeks. He had no fever, but the erythrocyte sedimentation rate was slightly increased (20 mm in the first hour). In addition there was a mild normochromic, normocytic anaemia (13 g/dl) and thrombocytosis (517,000/microliters). Gamma-globulin fraction was raised to 26%. The blood eosinophilia of 44% and the histological findings in a wedge biopsy of the lower leg (oedematous widened connective tissue septa, inflammatory infiltrate with eosinophilic granulocytes) established the diagnosis of eosinophilic fasciitis (Shulman syndrome). During oral treatment with prednisone (100 mg/d) the clinical symptoms regressed and the eosinophilia in peripheral blood disappeared. In the course of the subsequent seven months the prednisone dose was reduced gradually to 10 mg daily. But, because the symptoms did not entirely disappear the prednisone dose had to be increased temporarily for four times until there was a further, stepwise, reduction of the symptoms. But mild induration of the lower leg persisted. Maintenance treatment with prednisone, 10 mg daily, has been continued in order to avoid recurrence.

Adult↗

[Angiosarcoma following teratocarcinoma: "mutation", successive malignancy or irradiation].

Angiosarcoma is a rare malignant soft-tissue tumor and affects preferably skin and adjacent subcutaneous tissue. Very rarely the tumor is radiation-induced. We report the case of a 28-year-old Swiss who developed an angiosarcoma of the gut 5 years after the successful therapy of metastasising teratocarcinoma of his right testis by surgery and radiation therapy. We discuss three possible etiologies for the genesis of the angiosarcoma, which appeared rapidly after the primary tumor at an unusual site in an unusual patient.

Adult↗

Anatomy of the pelvic floor for translevatoric-transsphincteric operations.

The parasacral transsphincteric-translevatoric approach has proved of great interest in surgery of the lower rectum and urogenital organs. Due to this experience, anatomic specimens of the pelvic floor and pelvic organs were prepared in order to perfect the operative technique. The anatomic specimens show the pelvic floor and sphincter muscles and the blood supply by the pudendal vessels and nerve, as well as the topography of pelvic organs and fascias from the view of a surgeon performing parasacral surgery. The clinical experience with 118 cases of parasacral translevatoric-transsphincteric interventions at the Departments of Surgery of the University Hospital, Basel, and the Canton Hospital, Luzern, Switzerland, is presented. The results were mainly good. The operation technique is illustrated with selected cases of rectum resection, procedures for treatment of urethrorectal fistulas, reconstruction of pelvic floor for malformations, and reconstruction of injured urethra.

Abnormalities, Multiple↗

Effects of leukocyte interferon (E. coli) on human bone sarcoma growth in vitro and in the nude mouse.

Effects of highly purified human leukocyte interferon (rIFN-alpha 2) on colony formation, DNA synthesis and proliferation in nude mice of tumor cells from eight bone sarcomas have been studied. rIFN-alpha 2 produced a dose-dependent inhibition of [3H]thymidine incorporation by sarcoma cells. Even at high doses (10(4) U/ml), however, [3H]thymidine uptake could not be completely blocked by rIFN-alpha 2. In a cloning assay three established sarcoma cell lines and five other sarcoma samples obtained after short-term in vitro culture were found to be sensitive to various degrees to rIFN-alpha 2, complete inhibition being seen only at 10(4) U/ml. Three sarcomas were sensitive in the nude mouse model. Scheduling experiments revealed that rIFN-alpha 2 produces a delay in tumor growth only when administered either before or shortly after tumor implantation. Therefore rIFN-alpha 2 appears to be most active when tumor size is small and growth not exponential, indicating that rIFN-alpha 2 may play a role in an adjuvant setting. Growth sarcomas strongly suppressed by rIFN-alpha 2 in the cloning assay was markedly inhibited in the nude mouse. One sarcoma which was only moderately sensitive in the cloning assay was resistant in the animal experiment, confirming the predictive value of the clonogenic assay. Although the present findings demonstrate strong antitumor activity of rIFN-alpha 2 against human bone sarcoma cells they should be interpreted with caution mainly because the high rIFN-alpha 2 levels used in the experiments cannot be maintained in patients over a prolonged period.

Adolescent↗