17 March 2022: Textbook Updates

We have posted updates of the following topics:

Bone marrow neoplastic > Bone marrow – neoplastic myeloid > Myeloid / lymphoid neoplasms with eosinophilia and gene rearrangement > FGFR1 rearrangement
by Ahmed Samire Arfa, M.D., Ismail Elbaz Younes, M.D., Ling Zhang, M.D.
Topic summary: Pluripotent, hematopoietic, stem cell driven malignancy that may present as myeloproliferative neoplasm (MPN), acute myeloid leukemia (AML), T or B lymphoblastic lymphoma leukemia (T-ALL, B-ALL) or mixed phenotypic acute leukemia (MPAL). Presence of t(8:13)(p11.2;q12) or a variant of translocation leading to FGFR rearrangement in a myeloid progenitor, a lymphoblast or both. Age range of 3 – 84 years; median age of 32 years. Poor prognosis, even for patients presenting in the chronic phase due to the high incidence of transformation. Intensive chemotherapy with regimens such as hyper-CVAD for T or B cell lymphoblastic lymphoma, followed by early allogeneic transplantation, is recommended.

Bone marrow neoplastic > Bone marrow – neoplastic myeloid > Myeloid / lymphoid neoplasms with eosinophilia and gene rearrangement > PDGFRA rearrangement
by Ismail Elbaz Younes, M.D., Ling Zhang, M.D.
Topic summary: Myeloid, lymphoid or combined myeloid / lymphoid neoplasms often accompanied by eosinophilia and associated with FIP1L1-PDGFRA fusion, other PDGFRA gene rearrangements or an activating mutation of the PDGFRA gene. Can have different bone marrow pictures, most commonly chronic eosinophilic leukemia (CEL) or other diseases (e.g., acute transformation to acute myeloid leukemia or T cell acute lymphoblastic leukemia [T ALL]). Age range 7 – 77; mostly between 25 – 55. Good prognosis with absence of cardiac involvement. Treatment with imatinib even in cases presenting with acute myeloid leukemia or T cell acute lymphoblastic leukemia.

Bone marrow neoplastic > Bone marrow – neoplastic myeloid > Myeloid / lymphoid neoplasms with eosinophilia and gene rearrangement > PDGFRB rearrangement
by Ismail Elbaz Younes, M.D., Ling Zhang, M.D.
Topic summary: Myeloid or lymphoid neoplasm with frequent eosinophilia, sometimes neutrophilia or monocytosis; associated with rearrangement of PDGFRB, at chromosome 5, long arm (5q32). Can have different presentation; e.g., myeloproliferative neoplasm (MPN), myelodysplastic syndrome / myeloproliferative neoplasm (MDS / MPN), acute myeloid leukemia (AML), with MDS / MPN overlap syndrome being more common. Median overall survival is < 2 years before introduction of tyrosine kinase inhibitor; a case series of 26 cases treated with imatinib showed a longer survival (up to 6.6 years). Study showed a response rate to imatinib treatment of 96% with a 10 year overall survival rate of 90%.

CNS tumor > Meningeal tumors > WHO grading of meningiomas
by Jared T. Ahrendsen, M.D., Ph.D., Sanda Alexandrescu, M.D.
Topic summary: Family of neoplasms most likely derived from meningothelial cells of the arachnoid mater. Arabic numerals (1, 2, 3) now used instead of roman numerals (I, II, III). Molecular characteristics now part of the classification for anaplastic (malignant) meningioma, CNS WHO grade 3: TERT promotor mutation; CDKN2A/B homozygous deletion.

Penis & scrotum > Dysplasia / carcinoma in situ > Extramammary Paget disease
by Roberto Gonzalez, B.S., Debra L. Zynger, M.D.
Topic summary: Paget disease of the penis and scrotum is a rare, intraepidermal adenocarcinoma; it arises as a primary tumor or from secondary involvement of a nonpenoscrotal neoplasm. Surveillance, Epidemiology and End Results (SEER) registry reported 61% Caucasian and 36% Asian or Native American. Unfavorable prognostic factors: younger age, shorter symptom duration, delay in diagnosis, exudation, elevated serum CEA, adnexal involvement, greater depth of invasion, wide horizontal invasion, nodule formation, marked inflammation, lymphovascular invasion, HER2 / neu and p53 expression. Treatment: wide local excision or Mohs micrographic surgery.

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