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Biomedical subjects

H-J Holzhausen

Publications and source records attributed to H-J Holzhausen.

12 recordsLinked to original sources

[Primary nasal natural killer/T-cell lymphomas: classification and clinicopathological features].

BACKGROUND: Nasal NK/T-cell lymphomas are rare malignancies in Europe or North America. Histological diagnosis is difficult, because tumors imbedded in large necrotic areas and neoplastic infiltrates may be admixed with small lymphocytes, plasma cells, eosinophils, and histiocytes, and thus the process could be misdiagnosed as chronic inflammation. Progression of the disease leads to septal perforation and may also result in destruction of the hard palate, and if left untreated it ends fatally. This introduced the term "lethal midline granuloma", a term which should not be used any more. MATERIAL AND METHODS: Clinical features, pathohistology, and current classification of primary nasal NK/T-cell lymphomas are described against the background of the recent literature and a case report. RESULTS: Immunophenotyping is essential for the diagnosis. Tumor cells are uniformly infected by Epstein-Barr virus, which could be verified by EBER in situ hybridization. Immunohistochemically, tumor cells are positive for CD56, cytoplasmic CD3epsilon, and CD2 and they express cytotoxic molecules like granzyme B, TIA-1, GMP17, and perforin. Therapeutic options are radio- or radiochemotherapy. On average, 2- and 5-year survival rates of 50% are obtained in stages I and II. The prognosis of advanced tumor stages is very poor. CONCLUSIONS: Immunohistochemical and molecular genetic early diagnosis is of crucial prognostic relevance.

Adolescent↗

Expression of the stem cell self-renewal gene Hiwi and risk of tumour-related death in patients with soft-tissue sarcoma.

Self-renewal is considered as a common property of stem cells. Dysregulation of stem cell self-renewal is likely a requirement for the development of cancer. Hiwi, the human Piwi gene, encodes a protein responsible for stem cell self-renewal. In this study, we investigated the expression of Hiwi at the RNA level by real-time quantitative PCR in 65 primary soft-tissue sarcomas (STS) and ascertained its impact on prognosis for STS patients. In a multivariate Cox's proportional hazards regression model, we found that an increased expression of Hiwi mRNA is a significant negative prognostic factor for patients with STS (P=0.017; relative risk 4.6, 95% confidence interval (CI) 1.3-16.1) compared to medium expression of Hiwi transcript. However, a low expression of Hiwi transcript is correlated with a 2.4-fold (CI 0.7-8.0) increased risk, but this effect was not significant (P=0.17). Altogether, high-level expression of Hiwi mRNA identifies STS patients at high risk of tumour-related death. This is the first report showing a correlation between expression of a gene involved in stem cell self-renewal and prognosis of cancer patients.

Adult↗

[Morphological variability of synovial sarcoma in childhood].

We report on an unusual locally recurrent plantar soft tissue sarcoma in a 7-years-old boy. Due to an unusual morphology a clear diagnosis was initially not possible. The histologically different recurrent tumor was classified as synovial sarcoma by means of immunohistochemistry, ultrastructural and molecular analysis. This case demonstrates the variable morphological appearance of synovial sarcoma and the value of modern diagnostic procedures in such circumstances.

Antineoplastic Combined Chemotherapy Protocols↗

Prediction of mediastinal lymph node metastasis in medullary thyroid carcinoma.

BACKGROUND: Mediastinal lymph node metastases can be life threatening owing to their proximity to vital organs. Reliable identification of mediastinal metastasis is of utmost importance for timely mediastinal lymph node dissection, although suitable clinicopathological variables for their detection in patients with thyroid cancer have yet to be identified. METHODS: This was an analysis of 83 consecutive patients with radiological suspicion of mediastinal metastasis who underwent trans-sternal mediastinal lymph node dissection for node-positive medullary thyroid carcinoma between November 1994 and March 2003. RESULTS: Univariate analysis revealed that extrathyroidal extension (P < 0.001), distant metastasis (P = 0.001), the preoperative serum calcitonin level (P = 0.001), operation type (P = 0.004), contralateral cervicolateral metastasis (P = 0.016) and bilateral nodal metastasis (P = 0.031) were significantly associated with mediastinal involvement. Only extrathyroidal extension remained significant in a multivariate logistic regression analysis of mediastinal lymph node metastasis. Prediction of mediastinal metastasis by extrathyroidal extension was best at reoperation, with a specificity of 97 per cent and a positive predictive value of 88 per cent. CONCLUSION: Mediastinal lymph node dissection should be considered in patients undergoing reoperation for node-positive medullary thyroid carcinoma who have extrathyroidal extension and cervical lymph node metastases.

Adult↗

[Prolactinoma of the sphenoid sinus. Case report and literature review].

In this case study, we report on a patient complaining of headache who, after CT and MRI, was found to have a neoplasia of the left sphenoid sinus. After a transnasal biopsy and histological examination, a prolactinoma was diagnosed. Based on this case, we discuss important aspects of tumor biology, diagnostic procedures, histology as well as differential diagnosis. Prolactinoma has to be considered as a differential diagnose in all sphenoid sinus neoplasias with close contact to the pituitary gland.

Diagnosis, Differential↗

[Severe, non-infectious mitral valve endocarditis after mitral valve reconstruction in a 32-year old female with primary antiphospholipid syndrome].

BACKGROUND: A 32-year old female with primary antiphospholipid syndrome presented 8 months after mitral valve reconstruction with progressive exertional dyspnea and echocardiographically demonstrable critical mitral stenosis and regurgitation. Tachycardia, weight loss, sleep disturbances and increasing nervosity led to the diagnosis of concomitant hyperthyroidism. After the patient stopped the oral anticoagulation by herself, a 'catastrophic antiphospholipid syndrome' with multiple microthromboembolic events in several organs developed rapidly within a few weeks. Severe respiratory failure was observed 14 days after admission at our hospital because of a pulmonary edema. TREATMENT: Removal of the annuloplasty ring and alloplastic mitral valve replacement with a 25 mm bilifleat valve. Postoperatively, the patient was placed on oral anticoagulation. Several pre- and postoperative plasmaphereses lowered the level of antiphospholipid antibodies. The patient additionally underwent radioiodtherapy 5 months postoperatively. RESULTS: While hemodynamics and diuresis remained sufficient, ventilatory support with tracheostomy was necessary for 16 postoperative days to achieve stable respiration. Thirty months later, the patient is well and without further cardiac and neurological dysfunction. CONCLUSION: Secondary cardiac valve operations on patients with primary antiphospholipid syndrome may be successfully performed within a multidisciplinary approach. Oral anticoagulation remains the treatment of choice to prevent further thromboembolic events.

Adult↗

[Fibrous dysplasia of the petrous bone].

Fibrous dysplasia named after Jaffè and Lichtenstein is a benign, self-limiting disorder of the bone structure. The normal bone is replaced by cellular fibrous connective tissue. Fibrous dysplasia can be differentiated into monostotic and polyostotic forms and the McCune-Albright syndrome (combination of polyostotic areas in the bone with hyperpigmentation of the skin and endocrinological disturbances). The rare disease is predominantly found in the 1st to 3rd decade of life in the femur or humerus. In about 15% of all cases it affects the facial bones, and one-third of these is located in the jaw bone. Extremely rare is the invasion of the petrous bone. A 45-year-old female patient was examined because of tinnitus persisting for 1 year. In addition, she was suffering from increased hearing reduction and recurrent vertigo. The CT and MRT showed a milky glass-like tumor, which was limited by an osteolytic process situated in the left petrous bone. The process was removed via the mastoid. Histological examination revealed fibrous dysplasia. A highly differentiated osteosarcoma and an ossifying fibroma as important differential diagnoses have to be excluded because of the different therapeutic consequences.

Cerebellopontine Angle↗

Gains of 12q are the most frequent genomic imbalances in adult fibrosarcoma and are correlated with a poor outcome.

Comparative genomic hybridization was used to analyze 41 adult fibrosarcomas from 34 patients. Thirty-one patients showed in their tumors DNA sequence copy number changes (mean 11, range 3-25). The minimal common regions for the most frequent gains were narrowed down to 12q21 (18 cases); 12q14-q15 and 14q22 (16 cases each); 4q22, 7q31, and 14q23-q24 (15 cases each); and 4q21, 4q23-q24, 8q22, and 12q22 (14 cases each). Twenty-five high-level amplifications were observed in 12 samples. 12q21 and 18p were affected three times each; and 1p21, 4q31.3, 7p21, 12q14-q15, Xp22.1-p22.2, and Xq22-q23 two times each. Losses were less frequent than gains. Early stages of adult fibrosarcomas were characterized by frequent gains of chromosomes 2, 4q, and 14q, whereas gains of chromosomes 7 and 8q were associated with progression. Gains of 12q were frequent in all of the developmental steps of this soft-tissue sarcoma. By investigation of several tumors of the same patient, a number of corresponding changes were always detected. Adult fibrosarcomas from patients who died during the observation time showed statistically significant more frequent gains of 8q, 12q, 13q, and 15q compared to the fibrosarcomas of patients who are alive. Gains and high-level amplifications of 12q14-q22, which were the most frequent genomic imbalances, partly reflected an MDM2 amplification, indicating the importance of this region in the tumorigenesis of sarcomas. In adult fibrosarcomas, a gain of 12q22 correlated significantly (P = 0.028) with a poor overall survival rate.

Abdominal Neoplasms↗

[Merkel cell carcinoma].

Merkel cell carcinoma (cutaneous neuroendocrine carcinoma) is an uncommon, highly malignant, neuroendocrine skin tumour. Typically, the primary is a fast-growing tough dermal nodule that is characterized histologically by uniform round cells with a small cytoplasmic rim. The tumour cells express the cytokeratins 8, 18, 19, 20, neurofilament, synaptophysin, chromogranin, and neuron-specific enolase. A high frequency of local recurrences (25-77%) and lymph-node metastases (50%) are characteristic features of Merkel cell carcinoma. The 5-year survival rate is 30-74%. Merkel cell carcinomas are highly radiosensitive. Thus, besides surgical methods, radiation should be included into the treatment concept in every stage. We present four cases of Merkel cell carcinoma with different courses for a review-like discussion of this disease giving instructions for rapid diagnosis and effective therapy.

Aged↗

The hand-foot syndrome--a frequent secondary manifestation in antineoplastic chemotherapy.

The hand-foot syndrome (HFS) (palmoplantar erythrodysesthesia) designates acute, painful erythemas of the palms and soles of the feet caused by antineoplastic chemotherapies. The most frequent trigger substances are 5-fluoruracil and its derivates. At maximum severity, the HFS is bullous to erosive or ulcerous in character. The pathogenesis has not yet been clarified. Histologically, the HFS is characterized by a toxic keratinocyte reaction. Furthermore, there is sub-basal edema with a tendency to bullae, dilated blood and lymph capillaries and usually only mild perivascular lymphocytic infiltration. Early recognition and delineation from other differential diagnoses is prerequisite to targeted management of the disease. Depending on the severity, HFS requires dose reduction, interruption or switch in the antineoplastic chemotherapy.

Antineoplastic Agents↗