1 August 2022: Textbook Updates

We have posted updates of the following topics:

CNS & pituitary tumors > Gliomas, glioneuronal tumors, and neuronal tumors > Adult-type diffuse gliomas (topics to be merged) > Glioblastoma, IDH wild type
by Bharat Ramlal, M.D., Meaghan Morris, M.D., Ph.D.
Topic summary: An aggressive, infiltrating, astrocytic glioma that lacks mutations in IDH1, IDH2 and histone H3 genes. Characterized by diffusely infiltrative growth pattern with nuclear atypia and either histologically defined by brisk mitotic activity and microvascular proliferation or necrosis, or molecularly defined by the presence of TERT promoter mutation, EGFR amplification or combined gain of chromosome 7 and loss of chromosome 10. Poor prognosis, with a median survival of 8 months and 5 year survival rate of only 6.8%. Surgical resection where possible in younger patients (≤ 70 years old) and patients with good performance status, followed by radiotherapy with concurrent and adjuvant temozolomide (TMZ).

Lymph nodes & spleen, nonlymphoma > Lymph nodes-inflammatory / reactive disorders > Castleman disease
by Jayalakshmi Venkateswaran, M.D., Jayalakshmi Balakrishna, M.D.
Topic summary: Unusual heterogeneous group of lymphoproliferative disorders with some common morphological features involving lymph nodes or extranodal sites. 3 distinct clinical entities: unicentric Castleman disease (UCD), multicentric Castleman disease (MCD) and oligocentric or regional Castleman disease. UCD can affect all age groups and shows no gender difference with the median age of onset in the fourth decade. MCD affects patients of all ages with a median age of onset in the fifth to seventh decades. UCD: localized and excellent response to therapy. MCD: poorer prognosis, especially HIV positive patients and patients with MCD POEMS.

Ovary > Endometrioid tumors > Endometrioid cystadenoma / adenofibroma
by Stephanie L. Skala, M.D.
Topic summary: Benign tumors with endometrioid type glands, sometimes associated with endometriosis. Squamous or mucinous metaplasia or dystrophic stromal calcifications can be seen. In some cases, endometrioid cystadenomas may represent endometriotic cysts without obvious endometrial type stroma. Uncommon tumors, most often seen in postmenopausal women. Detected on pelvic imaging (ultrasound, CT, MRI). Symptoms may include abnormal vaginal bleeding, abdominal pain or pelvic mass. Treatment: not applicable; these tumors are benign.

Skin melanocytic tumor > Nevi > Dysplastic nevus
by Sepideh Nikki Asadbeigi, M.D., Cuong Nguyen, M.D.
Topic summary: Pigmented lesions that share clinical and histological features of both common nevi and melanoma. Although many melanomas arise de novo (without an obvious or detectable precursor lesion), approximately 25% histologically demonstrate an associated melanocytic nevi. Higher incidence among patients with history of melanoma. Diagnosis is heavily dependent on the histologic features and has a relatively poor interobserver correlation. Most dysplastic nevi never progress to melanomas even though they are associated with melanomas. Treatment: sun protection. Re-excision: decision to re-excise depends on the clinician’s perception and risk assessment.

Stains & CD markers > Tpit
by William McDonald, M.D.
Topic summary: Pituitary adenoma / pituitary neuroendocrine tumor has traditionally been classified using a combination of immunohistochemical stains for anterior pituitary hormones, including stains for prolactin, growth hormone, thyrotropin (TSH), luteinizing hormone (LH), follicle stimulating hormone (FSH), adrenocorticotropic hormone (ACTH) and the alpha subunit (ASU) of the glycoprotein hormones. Tpit is expressed within anterior pituitary gland corticotroph cells and within corticotroph adenomas. Nuclear expression is evaluated (reactivity only in the cytoplasm is regarded as negative). Untreated Cushing disease has high morbidity and mortality, with surgery being the treatment of choice for small, functioning corticotroph adenomas.

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