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[Clinical manifestation of Ewing's sarcoma as a retrotonsillar pharyngeal tumor].

BACKGROUND: Ewing's sarcoma is a malignant tumour which usually arises from bony structures. It can be found in dia- or metaphysis of bones of the extremities but as well in 50% of cases in skeletal parts of the trunk. Clinically patients often present with various symptoms as there are pain, swelling and progredient loss of function if extremities are involved. But in many cases, Ewing's sarcoma shows a lack of specific symptoms and therefore stays inapparent for a long time. CASE REPORT: A 14-year-old patient complained about intermittent nuchal pain and was sent to hospital because of an extensive retropharyngeal swelling. The preoperative imaging showed a tumour of the soft tissue of the neck. It had reached the cervical spine and after surgical treatment it was histologically and molecular biologically diagnosed as a Ewing's sarcoma arising from the transversal processes of the atlas. CONCLUSION: Since Ewing's sarcoma may have an immense component of soft tissue it has to be included in the range of differential diagnosis of soft tissue tumours of the head and neck, e.g., as there are extracranial schwannoma, inflammatory lymph nodes or lymph node metastases and lateral and medial cysts of the neck.

Adolescent↗

[Interesting case no. 45. Pharyngeal manifestation of chondroid chordoma].

Chordomas usually occur in the axial skeleton and they arise from the remnants of notochord. Their growth is slow and they often metastase. Most frequently chordomas are situated in sacral bone (50%), in spheno-occipital region of the skull base (35%) and in the cervical and lumbar spine (15%). We reported an unusual case of chordoma of the cervical spine with participation of the parapharyngeal space. The cervical chordoma was excised by total resection of the tumor mass with partial vertebrectomy C2/C3.

Aged↗

An approach for cure: PEI-chemotherapy and regional deep hyperthermia in children and adolescents with unresectable malignant tumors.

BACKGROUND: Elevated temperatures of 40 - 44 degrees C increase the actions of various anticancer drugs including N-lost derivatives, cytotoxic antibiotics and platinum analoga. In clinical usage thermochemotherapy (TCH) should facilitate surgical resection and ameliorate local tumor control. PATIENTS AND METHODS: From 07/1993 to 12/2002 a total of 39 patients have been enrolled onto a phase-II study (female = 24, male = 15, age 1 - 37.5 years, median 5.2). Among these, 24 patients had extracranial non-testicular germ cell tumors and 15 patients soft tissue or chondrosarcomas. INDICATION: locoregional relapse (n = 29) or unresectable tumor after neoadjuvant chemotherapy (n = 10). Among these two groups, there were ten patients with poor response or progressive disease under primary or relapse chemotherapy. Ten out of the 29 relapse patients had more than one relapse. Tumor site: pelvis (30), abdomen (4), head and neck (2), proximal leg (2) and lumbar spine (1). Thermochemotherapy (TCH): 1800 - 2000 mg ifosfamide/m (2) and 100 mg etoposide/m (2) on days 1 - 4 and 40 mg cisplatin/m (2) on days 1 + 4 combined with regional deep hyperthermia (42 - 44 degrees C, 1 h) on days 1 + 4. RESULTS: In 39 protocol patients a total of 166 TCH courses (332 heat sessions) were applied. 20 patients achieved complete response, and 10 patients achieved partial response. TCH was followed by surgical tumor resection in 28/39 patients and/or radiotherapy in 13/39 patients. At a median follow-up of 27 months, outcome in this high-risk patient population was 22 NED, 3 AWD, 12 DOD, 2 DOC. Five year event free (EFS) and overall survival (OS) for the whole study cohort was 0.39 +/- 0.11 (20/39 patients) and 0.52 +/- 0.11 (25/39 patients), respectively. CONCLUSION: TCH shows substantial therapeutic efficacy and facilitates complete tumor resection in 14 out of 28 operated patients. Multimodal treatment including TCH, surgical resection and/or radiotherapy leads to sustained remission in the majority of patients with locoregional tumor recurrence. The therapeutic effect is most pronounced, if TCH is administered at first relapse. Due to the clinical and histologic heterogeneity the number of patients eligible for TCH is limited. Therefore, a more valid assessment of treatment efficacy can only be made by a matched-pair comparison in cooperation with the clinical registers.

Abdominal Neoplasms↗

[Sacrococcygeal extraspinal myxopapillary ependymoma].

A 9-year-old boy was admitted with a circumscript subcutaneous tumor of the sacral region. Serum alpha-fetoprotein was not elevated. The lesion was excised en bloc including the top of the coccygis. Histology revealed a myxopapillary ependymoma without bony involvement. Metastases were excluded. Extraspinal myxopapillary ependymoma is a rare tumor of the sacrococcygeal region with a potential risk to develop metastases and for local relapse. Long-term follow-up is recommended.

Child↗

Intradural-extramedullary cavernous hemangioma of the left motor root C7--case report and update of the literature.

OBJECTIVE: Intradural-extramedullary cavernomas of the spine are rare lesions with only 21 published cases to date. Due to their rareness and special characteristics diagnosis often is difficult. We report on an additional case of an intradural-extramedullary cavernoma of the spine. PATIENT: A 56-year-old male presented with left shoulder pain and acute onset of pain affecting the whole spinal column two weeks prior to admission. There were no motor deficits, but a hypesthesia corresponding to the right distal C8-dermatome. MRI revealed an intradural-extramedullary, expansive lesion at the level of C6 with a hyperintense appearance in both T (1)- and T (2)-weighted images. Neither a hemosiderin rim nor contrast enhancement was visible. RESULTS: During surgery a hematoma and a reddish, berry-like tumor adherent to the left motor root C7 were removed. There were no new neurological deficits, and shoulder and back pain resolved within a few weeks after surgery. Histopathologically a cavernous hemangioma was diagnosed. CONCLUSIONS: The patient's symptoms were caused both by direct nerve compression and by spinal hemorrhage, most likely spinal SAH. As there was no characteristic hemosiderin rim and due to the hyperintense appearance in T (1)- and T (2)-weighted MR scans, a radiological diagnosis of hemorrhage and classification of the lesion was difficult. Despite their rareness, in patients with signs of spontaneous, spinal SAH and/or nerve compression syndromes cavernous hemangiomas have to be considered as a potential cause.

Hemangioma, Cavernous, Central Nervous System↗

[Differential diagnosis of spinal processes using modern diagnostic procedures].

Modern diagnostic management of diseases of the spinal cord is discussed, illustrated by some exemplary cases and reviewed by the literature. Special attention is directed to high resolution tomography, magnetic resonance imaging, analysis of the cerebrospinal fluid and electrophysiological methods. It seems conceivable that a shift from invasive methods to noninvasive procedures (e.g. magnetic resonance imaging) may occur.

Angiography↗

[Intraoperative ultrasound diagnosis in neurosurgery].

Report on 71 successful intraoperative sonographies. The advantages of the technique were particularly evident when searching for tumours which were not visible from the brain surface. In these conditions intraoperative sonography greatly facilitates the planning of the operative approach. The ultrasound examination allows to distinguish tumour from peritumoral edema and healthy tissues. In one case it proved superior to nuclear magnetic and computer tomography in determining extent and infiltration. The technique proved nearly indispensable for intraoperative puncture.

Brain↗

[Prenatal sonographic diagnosis of a cystic coccygeal teratoma with retroperitoneal extension].

The most common teratoma of th newborn is the sacrococcygeal, which contains tissues of all three germinal layers. It is located over and under the sacrum or the coccyx and may extend in front of the sacrum into the retroperitoneal region. The teratoma can lead to compression of the ureters with resultant hydronephrosis or to obstruction of the rectum. The most important complication is the malignant change which usually occurs between the fourth month and the third year. Because of the complications early diagnosis of the teratoma is necessary and extirpation should be carried out as soon as possible. The present paper reports on the sonographic findings in a sacrococcygeal teratoma with retroperitoneal extension at 37 weeks gestation. The aetiology and pathogenesis of this malformation and the clinical findings are discussed.

Adult↗

[Osseous metastases of a basalioma as a rare cause of sacral pain--case report].

Basal cell carcinoma-the most common malignant disease in man-only rarely metastasizes. The skeletal system is involved in 20 to 30% of the cases. As an isolated finding only a few cases are described. This case report presents the history of a 61 years old man, who developed 11 years after diagnosis of a basal cell carcinoma of the temple skin several vertebral metastases. Other sites of tumor manifestations could not be confirmed up to now. Clinical and histological findings as well as possible criteria for a high metastatic risk are discussed.

Basal Cell Carcinoma↗

Extracranial non-testicular teratoma in childhood and adolescence: introduction of a risk score for stratification of therapy.

PATIENTS AND METHODS: According to previous literature incomplete tumor resection, coccygeal or ovarian primary site and immaturity are known risk factors for relapse in teratoma. To establish a risk score points are allocated for resection, primary site and histology in the following manner and added: incomplete resection 4 points, primary site coccyx 3 points, ovary 2 points, other site 1 point, histological grading 0-3 points. This score system is evaluated on 270 extracranial non-testicular teratoma cases collected between 1982 and 1995 in the MAKEI cooperative treatment protocols of the German Society of Pediatric Oncology and Hematology. Treatment was resection alone (230 patients) or resection followed by postoperative adjuvant chemotherapy (40 patients). RESULTS: Patients treated with surgery alone: 28/230 (12%) patients relapsed, 14/230 (6%) patients showed highly malignant histology (mostly yolk sac tumor) in relapse. Mortality in case of relapse was 6/28 (21%). Patients scoring > or = 6 points (n = 45) had a relapse rate of 21/45 (47%) resulting in a 23%-mortality (5/21). Patients scoring < 6 points (n = 185) had a 4%-relapse risk (8/185) resulting in 13%-mortality (1/8) (p < 0.01). Patients treated with surgery and adjuvant chemotherapy: 7/40 patients (18%) suffered a relapse, none of them showing malignant histology. Mortality rate in case of relapse was 3/7 (43%). Patients scoring > or = 6 points initially treated with adjuvant chemotherapy (n = 18) had a relapse rate of 7/18 (39%), compared to patients scoring < 6 points (n = 22), in whom no relapses occurred (p < 0.01). There were no highly malignant relapses in the group treated with adjuvant chemotherapy. Regardless of the scored points the difference in highly malignant relapse histology comparing the group treated with surgery and adjuvant chemotherapy to the group treated with surgery was statistically significant (p = 0.02). CONCLUSION: The risk score system marks a high risk group including 63/270 (23%) of all evaluated extracranial non-testicular teratoma cases (scoring > or = 6 points). In this group 28/35 (80%) of relapses and 8/9 (89%) of tumor deaths occurred. For this high risk group a randomized trial will be suggested to evaluate the effect of adjuvant chemotherapy on the rate of malignant relapses. It should also be investigated, if adjuvant chemotherapy will influence relapse rate and mortality.

Child↗

Adult neuroblastoma: a report of two cases.

Neuroblastoma is an uncommon tumour arising from neural crest tissue. It is predominantly a tumour of childhood with a peak incidence in the first 3 years of life. In the USA the incidence is only about 500 paediatric cases per year. In adults it is an even more rare tumour. In 1986 Allan et al. spoke of a total of 39 reported cases of adult neuroblastoma in the world literature, with exception of some additional reports of cerebral neuroblastoma and peripheral neuro-epithelioma. Therefore neuroblastoma may cause both diagnostic and therapeutic problems. We report two recent cases of adult neuroblastoma.

3-Iodobenzylguanidine↗

[Skeletal involvement in malignant lymphoma].

Of 114 patients with Hodgkin's disease reviewed, 13 (11%) had bone involvement. The typical osseous lesion in Hodgkin's disease is a secondary manifestation in the axial skeleton. The lesions are mainly lytic; there is no relation between the site of the affection and the character of the lesion. Of 261 patients with non-Hodgkin lymphoma, 19 (7.3%) had bone involvement. Non-Hodgkin lymphoma very rarely affects the bone primarily; these lesions are lytic. Involvement of the appendicular skeleton is reported to by typical; this cannot be confirmed by our study. Most secondary osseous lesions in non-Hodgkin lymphoma are found in the cranium, the spine and the sternum; this pattern of distribution as well as the mainly lytic appearance can be considered typical.

Bone Neoplasms↗

[Primary bone tumors and tumor-like lesions of the spine. The accuracy of radiological assessment criteria].

In 79 primary bone tumors and tumorlike lesions of the spine radiological criteria are analyzed, which allow to assess the biological nature. Criteria associated with benign lesions, are: regular, trabecular matrix, osteoplastic matrix, exostotic growth, location in the posterior elements, absent soft tissue mass, and bulging of the vertebra. Criteria suggestive of malignancy are: Soft tissue mass, location inside the vertebral body, no alteration of the vertebral shape, compression fracture, and osteolysis. Benign lesions can be diagnosed with a higher degree of reliability than malignant tumors. Diagnostic accuracy increases in lesions exhibiting more than one criterion of the same group.

Chondroma↗

[The MRT demonstration of bone marrow changes in intervertebral disk degeneration].

Amongst 214 patients with degenerative disc disease, MRT showed focal fat deposition in the vertebral marrow close to the end plate in 87 (41%). The appearances and distribution of these zones is described. Recognition of these common changes is important in differential diagnosis between inflammatory and neoplastic processes.

Bone Marrow↗