4 August 2022: Textbook Updates

We have posted updates of the following topics:

CNS & pituitary tumors > Meningeal tumors > Anaplastic meningioma
by Valeria Barresi, M.D., Ph.D.
Topic summary: WHO 2021 definition: a meningioma with overtly malignant cytomorphology that can resemble a carcinoma, melanoma or high grade sarcoma, display markedly elevated mitotic activity, harbor a TERT promoter mutation or have homozygous CDKN2A / CDKN2B deletion. Occurs at any age, mainly adults 45 – 85 years. Sites: cerebral or spinal meninges; cerebral ventricles. Favorable prognostic factors: age 20 – 44 years and spinal location. Treatment: surgery followed by fractioned radiotherapy, experimental chemotherapy or peptide receptor radionuclide therapy.

Colon > Polyps > Inflammatory fibroid polyp
by M.J. Fernández-Aceñero, M.D., Ph.D.
Topic summary: Rare mesenchymal polypoid neoplasms that arise anywhere throughout the GI tract but most commonly in the stomach and small intestine. Rare in the large bowel. Predominance in middle aged adults (median 53 years), with wide variation (4 – 84 years). Discovery of overexpression and mutations in platelet derived growth factor receptor alpha (PDGFRA) has led to considering inflammatory fibroid polyp a neoplastic process. Good prognosis with no risk of malignization. Endoscopic resection is the preferred option, either polypectomy or endoscopic submucosal or radical resection.

Esophagus > General > Grossing & features to report
by Israh Akhtar, M.D.
Topic summary: This topic describes how to gross esophageal biopsies, esophagectomy specimens and endoscopic mucosal resection specimens. Location of biopsies: upper (cervical), middle (midthoracic) or lower esophagus (lower thoracic). Esophagectomies are performed with or without proximal stomach for severe dysplasia or for neoplasms; less frequently for strictures. If no gross tumor present (which is often the case after neoadjuvant therapy of the GE junction tumors), then the entire ulcerated / fibrotic area should be blocked off and submitted.

Pancreas > Neuroendocrine neoplasms > Neuroendocrine carcinoma
by Maria Gaia Mastrosimini, M.D., Claudio Luchini, M.D., Ph.D.
Topic summary: Poorly differentiated, high grade, malignant epithelial neoplasm with neuroendocrine differentiation. By morphology, PanNECs can be divided into small cell or large cell subtypes. Mean patient age is 59 years (patients are usually aged 50 – 60 years but can occur in younger patients as well). Very poor prognosis, compared to WD PanNETs; metastatic spread is present in most patients at the time of diagnosis. Treatment: surgical resection; chemotherapy (no established protocol) – platinum based regimen.

Soft tissue > Fibroblastic / myofibroblastic > Proliferative fasciitis
by Laura Warmke, M.D., Jeanne Meis, M.D.
Topic summary: Proliferative fasciitis is a pseudosarcomatous proliferation of myofibroblastic / fibroblastic cells with large ganglion-like cells involving subcutaneous tissue. Variable collagenous to myxoid stroma. Occurs predominantly in middle aged adults (mean age: 51 years). Subset of cases has a recurrent FOS gene rearrangement. Benign clinical course; usually self limited, disappearing within 1 – 12 weeks. Adequately treated by simple / conservative local excision.

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