The lymphoreticular system and bone marrow
C. Simon Herrington in Muir's Textbook of Pathology, 2020
Thymoma is a primary epithelial tumour of the thymus that behaves like a low-grade carcinoma, invading surrounding structures but seldom metastasizing (<10% of cases). The lesion consists of epithelial cells, often interspersed with so many thymic lymphocytes that it can be misdiagnosed as a lymphoma. Although often found incidentally, thymoma may be associated with myasthenia gravis, pure red cell aplasia, hypogammaglobulinaemia, and autoimmune diseases such as polymyositis. Several forms of lymphoma may affect the thymus, most notably classical Hodgkin lymphoma and mediastinal large B-cell lymphoma, both typically in young females. T-lymphoblastic leukaemia, which usually occurs in childhood, may present with a large anterior mediastinal mass. Germ cell tumours, similar to those found in the gonads, can arise at this site.
Introductory Remarks
Dongyou Liu in Tumors and Cancers, 2017
Tumors and cancers are usually named for the organs or tissues in which they start (e.g., brain cancer, breast cancer, lung cancer, lymphoma, skin cancer, etc.). Depending on the type of tissue involved, tumors and cancers are grouped into a number of broad categories: (i) carcinoma (involving the epithelium); (ii) sarcoma (involving soft tissue); (iii) leukemia (involving blood-forming tissue); (iv) lymphoma (involving lymphocytes); (v) myeloma (involving plasma cells); (vi) melanoma (involving melanocytes); (vii) central nervous system (CNS) cancer (involving the brain or spinal cord); (viii) germ cell tumor (involving cells that give rise to sperm or eggs); (ix) neuroendocrine tumor (involving hormone-releasing cells); and (x) carcinoid tumor (a variant of neuroendocrine tumor found in the intestine).
Gynaecological Problems – Answers
Rekha Wuntakal, Madhavi Kalidindi, Tony Hollingworth in Get Through, 2014
Germ cell tumours are common in young women and may produce tumour markers specific to their cell type. These include: AFP: endodermal sinus tumour and embryonal carcinomaβhCG: non-gestational choriocarcinomaβhCG: gestational trophoblastic diseaseβhCG may be raised: dysgerminomaPlacental alkaline phosphatase and lactate dehydrogenase (LDH): dysgerminoma (especially metastatic disease)Granulosa cell tumours: oestrogens and inhibinAndrogens (testosterone): Sertoli-Leydig cell tumours
Neoplasia in Turner syndrome: a retrospective cohort study in a tertiary referral centre in Belgium
Published in Acta Clinica Belgica, 2022
Cas Dejonckheere, Carolien Moyson, Francis de Zegher, Leen Antonio, Griet Van Buggenhout, Brigitte Decallonne
At present, the gene responsible for the increased risk of gonadoblastoma (hypothetically called GBY or gonadoblastoma locus on the Y chromosome) has not yet been identified. However, its location has been narrowed down to a 4 Mb pericentromeric region on the Y chromosome and it is known to be different from the SRY (sex-determining region of the Y chromosome) gene [33,34]. Several authors have suggested the TSPY (testis-specific protein Y-encoded) gene as a candidate for GBY [35,36]. Although its exact role remains unknown, it is believed to have a general promoting function in the development of germ cell tumours. In addition, prolonged expression of the embryonic germ cell marker OCT3/4 has been linked to malignant transformation. Detection of TSPY, OCT3/4, and other germ cell markers allows to characterise the disturbed process of germ cell maturation on biopsies of dysgenetic gonads. Another important factor in the pathway leading to germ cell tumour development is stem cell factor (SCF). This marker is consistently detected in gonadoblastoma and can be used to differentiate from simple maturation delay. The exact tumour pathogenesis thus proves to be complex, involving a subtle interplay of genetic susceptibility, epigenetic, and microenvironmental parameters (e.g. change in endocrine environment) [9,37,38]. Efforts should be made to send pathology specimens to well-equipped, specialised centres for expert diagnosis, in order to maximise information on the presence of (pre)malignant germ cells and thus improve individual risk assessment.
Balancing efficacy with long-term side-effects: can we safely de-escalate therapy for germ cell tumors?
Published in Expert Review of Anticancer Therapy, 2023
The long-term effects of chemotherapy and/or radiotherapy in managing germ cell tumors are significant. Minimizing the number of cycles given and avoiding unnecessary irradiation are important in minimizing long-term effects. Changes in practice which would reduce the risk of long-term effects are possible. The rarity of germ cell tumors and the concerns that any change in treatment may compromise the current excellent disease control are hampering the introduction of newer treatments in situations where definitive trials are unlikely. The introduction of miRNA technology in monitoring risk of relapse is likely to lead to more personalized treatment based on an individual’s response to therapy. The use of RPLND as a chemotherapy-sparing approach warrants further investigation. Extended follow-up is needed if any new approaches are necessary in case unanticipated late effects occur.
Metastatic testicular choriocarcinoma presenting as eyelid swelling and ptosis
Published in Orbit, 2022
Tharsica Sivagnanasithiyar, Linda Okafor, Guy Faust, Hardeep Singh Mudhar, Samantha Sii, Christopher Knapp, Raghavan Sampath
Neoplasms of the testes only constitute 1% of all malignant tumours in men and most commonly affect younger age groups.4 Testicular cancers may be categorized into germ cell tumours and non-germ cell tumours. Of the germ cell tumours (95%) which comprise several subtypes, they may be broadly split into seminoma and non-seminomatous germ cell tumours with choriocarcinomas being the rarest and most aggressive form. In males’ choriocarcinomas are typically mixed germ cell in origin.4,5 Testicular choriocarcinoma is a rare cause of metastases to the ocular components and a review of the literature was performed identifying a handful of cases mostly with choroidal metastases (supplementary table 1). Drami-Gavazi et al. reported a case of gestational choriocarcinoma metastasizing to the medial rectus which was first case report of its kind.6
Related Knowledge Centers
- Benign Tumor
- Birth Defect
- Gonad
- Ovary
- Embryo
- Neoplasm
- Germ Cell
- Cancer
- Testicle
- Developmental Biology