S1 Guidelines for the Kaposi Sarcoma.

Kaposi's sarcoma (KS) is a rare, malignant, multilocular vascular disease originating from lymphatic endothelial cells that can primarily affect the skin and mucous membranes, but also the lymphatic system and internal organs such as the gastrointestinal tract, lungs or liver. Five epidemiological subtypes of KS with variable clinical course and prognosis are distinguished, with increased incidence in specific populations: (1) Classical KS, (2) Iatrogenic KS in immunosuppression, (3) Endemic (African) lymphadenopathic KS, (4) Epidemic, HIV-associated KS and KS associated with immune reconstitution inflammatory syndrome (IRIS), and (5) KS in men who have sex with men (MSM) without HIV infection. This interdisciplinary guideline summarizes current practice-relevant recommendations on diangostics and therapy of the different forms of KS. The recommendations mentioned in this short guideline are elaborated in more detail in the extended version of the guideline (online format of the JDDG).

S1-Leitlinie: Kaposi-Sarkom.

Das Kaposi-Sarkom (KS) ist eine seltene, maligne, von lymphatischen Endothelzellen ausgehende, multilokuläre Gefäßerkrankung, die vor allem Haut und Schleimhäute, aber auch das lymphatische System und innere Organe wie den Gastrointestinaltrakt, die Lunge oder die Leber befallen kann. Fünf epidemiologische Subtypen des KS mit variablem klinischem Verlauf und unterschiedlicher Prognose werden unterschieden, die in spezifischen Populationen vermehrt auftreten: (1) klassisches KS, (2) iatrogenes KS bei Immunsuppression, (3) endemisches (afrikanisches) lymphadenopathisches KS, (4) epidemisches, HIV-assoziiertes KS und mit einem Immunrekonstitutions-Inflammations-Syndrom (IRIS) assoziiertes KS und (5) KS bei Männern, die Sex mit Männern haben (MSM) ohne HIV-Infektion. Diese interdisziplinäre Leitlinie fasst aktuelle praxisrelevante Empfehlungen zu Diagnostik und Therapie der verschiedenen Formen des KS zusammen. Die in dieser Kurzleitlinie genannten Empfehlungen werden in der Langfassung der Leitlinie (Online-Version des JDDG) detaillierter ausgeführt.

Optical coherence tomography: its role in daily dermatological practice.

Optical coherence tomography (OCT) is a non-invasive, tomographic imaging technique which generates high-resolution in-vivo images up to mid-dermal layers. Due to continuous technological improvements, OCT is moving from research projects into daily dermatological practice. It can complement other imaging methods like high-frequency ultrasound or confocal microscopy. There is a wide variety of indications for OCT. In addition to aiding in the diagnosis and clinical monitoring of inflammatory dermatoses, OCT is a very useful and feasible technique in dermato-oncology.

[Differential diagnoses of benign eyelid tumors in children and adolescents]. / Differenzialdiagnosen benigner Lidtumoren bei Kindern und Jugendlichen.

BACKGROUND: The clinical diagnosis as well as the treatment approach of periocular tumors in childhood and adolescence can be challenging. Knowledge of the most important differential diagnoses and their clinicopathological correlation is helpful for the treatment approach. OBJECTIVE: The clinical and histological characteristics of various eyelid tumors in childhood and adolescence are presented taking the excision frequencies into consideration. MATERIAL AND METHODS: The frequencies and clinicopathologic correlation of the most important eyelid tumors (n = 485) are presented based on the data of the ophthalmopathology laboratory of the University Eye Hospital Bonn from 1998-2023. RESULTS: The most frequent tumor in childhood and adolescence is chalazion (57.3%), followed by dermoid cysts (16.7%) and molluscum contagiosum (9.6%). Other lesions of childhood and adolescence include pilomatrixoma (2.1%), hemangioma and other vascular malformations (4.7%) and rare differential diagnoses, such as subcutaneous calcifying nodules and xanthogranuloma. Guidance on the approach in different age groups is presented in the form of a decision tree. CONCLUSION: Tumors in children and adolescents are mostly benign, yet there are important indications for excision. A histological examination of any excised tissue in childhood and adolescence is obligatory because unexpected findings are not uncommon and the spectrum of lesions also differs from that in adulthood. Knowledge of the histological picture can be very helpful in the preoperative clinical classification and for planning further procedures.

Risk factors for relapse in clinical stage I nonseminomatous testicular germ cell tumors: results of the German Testicular Cancer Study Group Trial.

PURPOSE: To prospectively assess potential risk factors for relapse in clinical stage I nonseminomatous germ cell tumors of the testis (CS I NSGCT). PATIENTS AND METHODS: From September 1996 to May 2002, 200 patients with CS I NSGCT were prospectively assigned to retroperitoneal lymph node dissection (RPLND), and risk factor assessment was performed within a multicenter protocol. One hundred sixty-five patients had an adequate minimum follow-up of 12 months (mean, 34.5 months) or had pathologic stage II. RESULTS: Pathologic stage II disease was found in 27.9% of patients. Only 0.6% of patients relapsed in the retroperitoneum after confirmation of pathologic stage I disease. With reference pathology, vascular invasion (VI) was most predictive of stage in multifactorial analysis (accuracy, 65.1%). However, the positive predictive value (PPV) of VI to predict patients who have metastatic disease or relapse during follow-up was only 52.7%. With absent VI, low-risk patients had a negative predictive value (NPV) of 76.9%. With a combination of several risk factors, the PPV increased to 63.6% and the negative predictive value increased to 86.5%. CONCLUSION: Even with an optimal combination of prognostic factors and reference pathology, more than one third of patients predicted to have pathologic stage II or relapse during follow-up will not harbor metastatic disease and, therefore, would be overtreated with adjuvant therapy. However, patients at low risk may be predicted at an 86.5% level, and thus, surveillance in highly compliant patients would be a valuable option. For high-risk patients, further reduction of adjuvant treatment is necessary.

Comparative morphometric analysis of primary versus recurrent basal cell carcinoma and of histological subtypes. Significance of morphometry of the nuclei.

BACKGROUND: Basal cell carcinomas (BCCs) are the most common cutaneous tumors, accounting for approximately 70% of all malignant skin tumors. A morphometric analysis was performed to evaluate whether morphometric parameters of the nuclei of BCC tumor cells may serve as predictors for biological behavior like recurrence rate or are able to differentiate morphological subtypes (multifocal superficial, nodular, sclerosing and miscellaneous forms of BCC). MATERIALS AND METHODS: N = 130 histological sections from n = 126 patients with BCC of the head and neck were analyzed. Morphometry included: maximum and minimum diameter, perimeter, nuclear area (direct parameters). According to the direct parameters, so called form factors were evaluated: axis ratio, shape factor, nuclear contour index, nuclear roundness and circumference/contour ratio. RESULTS: Morphometric parameters were not able to differentiate significantly between primary and recurrent BCC. Concerning the histological subtypes, multifocal superficial BCC differed significantly (< 0.05) from all other subtypes by direct parameters. CONCLUSION: Morphometry can help to distinguish between histological subtypes of BCC. Morphometry alone is not able to predict recurrence rate.

[Fatal outcome of Ecstasy overdose]. / Todesfall nach Ecstasy-Uberdosierung.

Consumption of amphetamine derivatives has considerably increased in Germany since the early nineties. Again and again intoxications with lethal outcome have also been reported, especially after physical activities such as intensive dancing. The authors present a case of an obviously suicidal intoxication of a 21-year-old man who was found dead with marked cuts on the right forearm. Toxicological tests showed in particular 3, 4-methylene-dioxymethamphetamine (MDMA). The results of the hair analysis revealed chronic consumption, but no cellular liver damage could be demonstrated. When examining the body fluids and organs, the highest concentrations by far were measured in the lungs (36.6 mg/kg), the liver (29.7 mg/kg) and the brain (29.1 mg/kg). The concentration in heart blood amounted to 10.8 mg/kg and was thus markedly higher than in peripheral blood (7.2 mg/kg). In the muscles concentrations ranged between 14.3 mg/kg and 20.2 mg/kg. On the basis of these concentrations and the available pharmacokinetic data the amount of MDMA probably consumed is assessed. It is demonstrated that for this assessment the concentrations in the muscular system are of special importance, as redistribution of highly lipophilic substances from the surrounding tissue is possible also in peripheral blood.

Mondor's disease: a rare cause of anterior chest pain.

We report the case of a 52-year-old patient who sought a rheumatologic consultation for anterior chest pain of 2 weeks' duration. Radiology for trauma did not reveal any pathology. The laboratory findings focusing on rheumatologic diseases remained unhelpful and no abnormalities were found. After a cutaneous linear cordlike lesion at the right lateral chest wall occurred, the diagnosis of Mondor's disease could be made on the basis of clinical and histologic investigation. Because pain precedes the typical clinical manifestation, problems of diagnosis can occur in the early phase of the illness.

c-kit mutations in gastrointestinal stromal tumors occur preferentially in the spindle rather than in the epithelioid cell variant.

Gastrointestinal stromal tumors (GISTs) coexpress CD34 and the Kit tyrosine-kinase receptor (CD117). A subset of GISTs carry gain-of-function mutations of the c-kit proto-oncogene in its juxtamembrane domain. The relationship between the mutational status and histological as well as immunohistochemical features has not been assessed in detail. 36 GISTs and 14 other gastrointestinal mesenchymal tumors were investigated for their morphology and immunophenotype as well as for the presence of c-kit mutations. DNA was extracted from formalin-fixed, paraffin-embedded tissue. Exons 9, 11, 13, and 17 of c-kit were analyzed by SSCP. Bands with altered mobility were excised, reamplified, and sequenced. C-kit mutations in Exon 11 encoding the juxtamembrane domain were identified in 19 cases (52.8%), with deletions in 12 cases, insertions in 3 cases (2 of these as duplications), and point mutations in 4 cases. The mutations clustered between Codons 553 and 561, pinpointing the critical region for deregulated Kit receptor activation. In both Exons 9 and 13, single mutations could be identified, whereas no mutations were found in Exon 17. There were c-kit mutations in 66.6% of benign GISTs (14/21), 83.3% of the malignant (5/6), and 40% of the cases of intermediate malignancy (2/5). A low frequency of mutations in benign GISTs, as reported previously by other researchers, could not be observed in our panel. Interestingly, all GISTs with c-kit mutations displayed a spindle cell phenotype, whereas mutations were absent in all 7 tumors with an epithelioid component (P =.03). This finding suggests a relationship between c-kit mutation and histological subtype in GISTs.

Deletion of Trp-557 and Lys-558 in the juxtamembrane domain of the c-kit protooncogene is associated with metastatic behavior of gastrointestinal stromal tumors.

Gastrointestinal stromal tumors (GISTs) typically express high levels of the Kit-receptor. The majority of GISTs carry mutations in the c-kit protooncogene clustering in exon 11. The significance of c-kit mutations for the biological behavior of GISTs is still under discussion. We evaluated 55 sporadic GISTs with available follow-up data for c-kit mutations in the juxtamembrane domain and detected mutations in 35 cases (63.6%). We found a mutational hotspot in codons 557 (tryptophan) and 558 (lysine) preferentially in histomorphologically malignant tumors. In the group of GISTs carrying c-kit mutations, 16 of 21 malignant, but only 3 of 8 benign GISTs and 3 of 6 lesions with uncertain malignant potential, carried mutations of Trp-557 and/or Lys-558. We investigated whether mutations in these 2 amino acids had an impact on biological behavior. Trp-557 and/or Lys-558 were mutated in all 15 metastatic GISTs carrying c-kit mutations but only in a minority of nonmetastatic tumors. A combined deletion of Trp-557 and Lys-558 occurred exclusively in 8 metastatic GISTs. We conclude that in addition to histomorphological evaluation determination of mutations in exon 11 may be an additional parameter for predicting the metastatic risk of GISTs and may be important for the decision that patients will need close clinical follow-up or further adjuvant treatment with kit antagonists.