Pathologie (Heidelberg, Germany)最新文献

Neuroendocrine tumors (NETs) are a diverse group of neoplasms that originate from neuroendocrine cells throughout the body. Diagnosing NETs presents unique challenges due to their varied presentation, morphology, and biological behavior. This article provides an overview of the key diagnostic principles relevant to general pathologists, emphasizing the importance of a multidisciplinary approach that integrates clinical, radiological, histopathological, and immunohistochemical data. The emergence of new markers as well as recent advances in molecular pathology and the application of grading systems are discussed, highlighting their impact on prognosis and therapeutic strategies.

Background: Besides reactions of the IgE-mediated immediate type, medicamentous therapies can cause a variety of different mucocutaneous adverse events. Exanthematous manifestations require a fast and certain diagnosis due to their extent, sometimes rapid progression, and mucous membrane or organ involvement.

Objectives: The spectrum of non-IgE-mediated exanthematic drug reactions is covered.

Material and methods: The most relevant reactions are portrayed clinically and histopathologically.

Results: Displayed are classical maculo-papular drug eruption, lichenoid drug reaction, acute generalized exanthematic pustulosis (AGEP), severe potentially life-threatening drug reactions such as Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) as well as generalized bullous fixed drug eruption (GBFDE), drug reaction with eosinophilia and systemic symptoms (DRESS), and some others.

Conclusions: Cutaneous drug-related side effects cover a broad spectrum. Important for the correct treatment is a reliable diagnosis. In the case of severe, life-threatening drug reactions, however, permanent discontinuation of the drug is essential.

Background: Lymphangioleiomyomatosis (LAM) is a rare, slow progressing, low-grade neoplasia that primarily effects young women. The disease is well known for its pulmonary involvement with cystic destruction, but extra-pulmonary disease may occur. LAM is associated with mutations in the TSC 1 or TSC 2 genes and may develop sporadically or in the context of hereditary disease tuberous sclerosis complex (TSC). Incident LAM may represent the sentinel finding of the disease.

Objective: Raising awareness for rare extrapulmonary LAM lesions in retroperitoneum and pelvic cavity.

Methods: Data-based research was performed for LAM in gynecological surgical specimens. H&E-stained slides were re-examined, and immunohistochemical stains were re-evaluated. Clinical data were retrieved for the presence pulmonary LAM or TSC.

Results: A total of 13 cases were identified. The age of the patients ranged from 32 to 77 years, and 8/13 were ≤ 55 years. Two women had a history of pulmonary LAM and TSC. Most women underwent surgery for gynecological malignancy. On histological examination, 10/13 patients presented LAM in 1 to 9 lymph nodes with a lesion size of 0.5 to 12.0 mm, mainly located subcapsular or in the nodal parenchyma. Three of the 13 women showed extranodal involvement of the retroperitoneum, myometrium, and the hilum of the ovary. Immunohistochemically LAM was positive for HMB45, desmin, and smooth muscle actin.

Conclusion: LAM is a rare systemic disease that mainly involves the lungs. Nevertheless, extra-pulmonary manifestations may occur. It is important to report the incidental finding of even small foci of LAM within the pathology report. Incidental LAM may represent the sentinel lesion for pulmonary LAM and/or TSC.

The article presents the concept of misdiagnosis or malpractice to practising pathologists from a medical and legal perspective and highlights similarities and differences in this respect. In particular, the risk of criminal liability for pathologists in the context of their practice activities is addressed and the relationship between the legal judgements of criminal, civil and professional law is examined in more detail. Due to the lack of an existing reversal of the burden of proof in criminal law, the constellation may arise in practice that a treatment error under civil law is assumed. However, such a qualification does not necessarily lead to a criminal accusation. Furthermore, the field of professional law, which is often pushed into the background for practising lawyers, is also emphasised. Criminal sanctions often hit a practitioner less severely than, for example, the withdrawal of a licence to practise under professional law. Close dovetailing of criminal, civil and professional law advice is essential in this respect. Pathologists are also given tips on how to behave based on many years of criminal defence practice in order to enable an adequate defence at an early stage in the event of a confrontation with criminal charges.

The 5th Edition of the "WHO Classification of Tumours: Urinary and Male Genital Tumours" introduces several significant updates to the classification of testicular tumours. These updates include revised terminology for special germ cell tumour subtypes (neuroectodermal and neuroendocrine tumours) of the testis. Additionally, the signet-ring stromal tumour and myoid gonadal stromal tumour have been introduced as distinct entities within the sex-cord stromal tumours. Moreover, new combined sections have been created for lymphatic neoplasms and soft tissue tumours of the urinary and male genital tract.

Histological subtyping of diverse renal cell carcinomas (RCCs) has seen significant changes during the last two decades. This resulted in the introduction of several new phenotypically and genetically defined entities, many which are also listed in the current WHO classification. Some of these well-defined entities may, under certain circumstances, undergo a process of dedifferentiation resulting in loss of their phenotypic and immunohistochemical features, hence adopting a non-descript anaplastic morphology. Accordingly, the original entity-defining tumor clone might be either totally overgrown and lost or just be missed by sampling the tumor. This final common pathway of dedifferentiation results in several oncological disadvantages and prevents a histology-tailored approach to systemic therapy. In addition, the possibility of inherited cancer as in the case of SDH- and FH-deficient RCC would be easily overlooked if the exact subtyping is not possible. This overview article illuminates the main RCC subtypes that may undergo dedifferentiation and their differential diagnostic approach.