31 March 2022: Textbook Updates

We have posted updates of the following topics:

Bone marrow neoplastic > Bone marrow – neoplastic myeloid > Other AML entities defined by the WHO > AML with myelodysplasia related changes
by Srishti Gupta, M.B.B.S., M.D., Elizabeth Courville, M.D.
Topic summary: ≥ 20% myeloid blasts in the blood or bone marrow with 1 or more of the following: features of myelodysplasia, prior history of myelodysplastic syndrome (MDS) or MDS / myeloproliferative neoplasm (MPN) or MDS related cytogenetic abnormalities. Commonly presents with severe pancytopenia. Generally poorer prognosis with lower remission rates and shorter overall survival than other AML subtypes. Diagnosis of AML-MRC solely based on multilineage dysplasia or previously untreated MDS or MDS / MPN have significantly better outcomes than those with MDS defining cytogenetic abnormalities or disease secondary to a previously treated MDS or MDS / MPN.

Eye > Conjunctiva > Epithelial tumors > Conjunctival intraepithelial neoplasia
by Nfn/Fnu Kiran, M.D., Pia Mendoza, M.D.
Topic summary: Spectrum of abnormal growth of atypical squamous epithelial cells of the conjunctiva; graded based on thickness of epithelial involvement. Estimated incidence is 17 – 20 cases per 1 million person years. Incidence rate varies worldwide; highest rates in Africa (3.4 cases per 100,000 per year in Zimbabwe). Appears to be multifactorial, involving a variety of environmental factors in a susceptible host. Recurrence associated with higher grade lesions, tumors, advanced American Joint Committee on Cancer (AJCC) T category and positive margins. Treatment: surgical excision using a no touch technique with 2 – 4 mm margins, alcohol application and supplemental surgical margin cryotherapy.

Kidney tumor > Benign adult tumors > Juxtaglomerular cell tumor
by Alcino Pires Gama, M.D., Bonnie Choy, M.D.
Topic summary: Rare renin secreting tumor arising from the specialized smooth muscle cells of the glomerular afferent arteriole in the renal juxtaglomerular apparatus. Frequently associated with severe and poorly controlled hypertension, hypokalemia and hyperaldosteronism secondary to tumor renin secretion. Seen in young adults (20 – 35 years) but has been reported in children and older adults. 90% of patients will become normotensive following tumor resection. 10% remain mildly hypertensive despite surgical resection. Treatment: surgical resection – partial or total nephrectomy.

Pancreas > General > Papanicolaou system
by Victoria Jones, M.S., Derek Allison, M.D.
Topic summary: Internationally adopted recommendations for sampling, diagnosing and managing pancreaticobiliary cytology lesions. 6 tiered categorical system resulting in overall improved sensitivity / specificity and risk stratification. Primary method for sampling solid mass and cysts: endoscopic ultrasound fine needle aspiration (EUS FNA). Primary method for sampling strictures: endoscopic retrograde cholangiopancreatography (ERCP) guided brush cytology. Risk of malignancy (ROM) = total number FNA specimens that proved malignant on follow up / total number of surgical resections and clinical follow ups.

Testis & paratestis > Sex cord stromal tumors > Large cell calcifying Sertoli cell tumor
by Stephanie Siegmund, M.D., Ph.D., William Anderson, M.D., Andres Acosta, M.D.
Topic summary: Large cell calcifying Sertoli cell tumor (LCCSCT) was first described by Lange et al. in 1960. Generally presents as a benign sex cord stromal tumor found predominantly in patients < 20 years old; a subset presents in older patients and may behave aggressively. 20 – 40% of cases associated with Carney complex due to mutations in PRKAR1A gene. Kratzer et al. proposed malignant prognostic features that include age > 25 years old and 2 or more of the following adverse features: size > 4 cm, extratesticular extension, mitotic index > 3/10 high power fields (HPFs), coagulative tumor necrosis, vascular invasion, high grade cytologic atypia. Treatment: partial or radical orchiectomy for organ confined disease.

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