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Endocrine System

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Pathology of Childhood and Adolescence

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Abstract

The endocrine system pathology will primarily discuss the molecular and surgical pathology of the adrenal gland and thyroid gland. In the adrenal gland, text and illustrative material will focus on neuroblastoma, and ganglioneuroblastoma, as well as pheochromocytoma and adrenocortical carcinoma. Thyroid cancer is the wildest growing cancer in women. Although papillary thyroid carcinoma, which makes up about 85% of all thyroid gland cancer, has an excellent prognosis, some variants, such as tall cell and insular variants, have shown an aggressive outcome. Thyroid pathology will include cytology photographic material, benign and malignant carcinomas. There will be a section also for the diffuse sclerosing variant of papillary thyroid carcinoma, which constitutes about 1–2% of all cancers with a preference for young female children and adolescents and has been associated with a poor prognosis.

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Authors and Affiliations

  1. University of Alberta, Edmonton, AB, Canada

    Consolato M. Sergi

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  1. Consolato M. Sergi
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Correspondence to Consolato M. Sergi .

Multiple Choice Questions and Answers

Multiple Choice Questions and Answers

  • END-1 What is splenogonadal fusion?

    1. (a)

      A congenital defect with an abnormal connection between the primitive spleen and gonad that occurs during gestation

    2. (b)

      A gene fusion transcript involving the expression of genes of the spleen and the gonads

    3. (c)

      A defect involving the genes responsible for the expression of the splenium of the corpus callosum and the developmental genes of the testis

    4. (d)

      A combined defect involving the genes responsible for the expression of the splenium of the corpus callosum and the developmental genes of the ovary

  • END-2 Which of the following cytologic features does NOT belong to a papillary thyroid carcinoma?

    1. (a)

      “Salt-and-pepper” chromatin

    2. (b)

      Grooves

    3. (c)

      Pseudo-inclusions

    4. (d)

      Nucleolus

    5. (e)

      Thickening of the nuclear membrane

    6. (f)

      Irregularity of the nuclear membrane

  • END-3 Some parents are concerned that their 15-year-old child did not start to menstruate. Although in some occasions menstruation may be delayed in healthy children, the child showed short stature, webbing of the neck, and cubitus valgus at the physical examination. Intellectually her school performance was unremarkable. Which of the following investigations will be appropriate at first?

    1. (a)

      Serum long-chain fatty acids

    2. (b)

      Organic acid analysis of urine

    3. (c)

      Muscular biopsy

    4. (d)

      Computed tomography of the head with a primary focus on the sella turcica

    5. (e)

      Karyotype

  • END-4 A 17-year-old girl develops a unilateral enlargement of her thyroid gland, and the lump undergoes a fine needle aspiration cytology (FNAC), which is shown here:

figure b

  • What is the most likely diagnosis?

    1. (a)

      Papillary thyroid carcinoma

    2. (b)

      Follicular thyroid carcinoma

    3. (c)

      Hashimoto thyroiditis

    4. (d)

      Benign nodule

    5. (e)

      Graves disease

  • END-5 Which of the next features does NOT help in distinguishing a parathyroid carcinoma from parathyroid adenoma?

    1. (a)

      Capsular invasion

    2. (b)

      Vascular invasion

    3. (c)

      Metastases

    4. (d)

      Pleomorphism

  • END-6 Which of the next statements regarding the Smith-Lemli-Opitz syndrome (SLOS) is NOT correct?

    1. (a)

      It is an abnormality in cholesterol metabolism resulting from deficiency of the enzyme 7-dehydrocholesterol (7-DHC) reductase.

    2. (b)

      The serum concentration of cholesterol is usually low.

    3. (c)

      DHCR7 gene encodes the 7-DHC reductase.

    4. (d)

      The inheritance pattern is autosomal dominant.

    5. (e)

      Infants with SLOS show prenatal/postnatal growth retardation, microcephaly, intellectual disability, distinctive facial features, cleft palate, cardiac defects, the underdeveloped external genitalia (males), postaxial polydactyly, and 2–3 syndactyly of the toes.

  • END-7 A 1-year-old infant present with neuroblastoma and the anatomo-pathologic diagnosis with mitotic karyorrhectic index (MKI), MYCN status, and staging is presented at the pediatric tumor board. Which of the following features that need to be discussed is associated with unfavorable histology in a patient of this age?

    1. (a)

      Ganglioneuroblastoma, intermixed

    2. (b)

      Differentiating neuroblastoma, high MKI

    3. (c)

      Poorly differentiated neuroblastoma, intermediate MKI, MYCN non-amplified

    4. (d)

      Hyperdiploid neuroblastoma

    5. (e)

      Nodular ganglioneuroblastoma with intramodular low MKI value

  • END-8 Which neoplasm reveals a positivity for NSE in tumor cells, negativity for AE1–AE3 (low and high molecular weight keratin group) in tumor cells, and S100 positivity in interstitial (“sustentacular”) cells?

    1. (a)

      Alveolar soft part sarcoma

    2. (b)

      Pheochromocytoma

    3. (c)

      Malignant melanoma

    4. (d)

      Malignant peripheral nerve sheath tumor

  • END-9 Which neoplasm may exhibit mutations in succinate dehydrogenase subunit genes of the mitochondrion?

    1. (a)

      Metastatic rhabdomyosarcoma

    2. (b)

      Malignant melanoma

    3. (c)

      Glioma

    4. (d)

      Malignant peripheral nerve sheath tumor

    5. (e)

      Pheochromocytoma

  • END-10 The immunohistochemistry plays a significant role in the diagnosis of adrenal gland masses. Which immunohistochemical stain of the following list, if positive, would predilect a diagnosis of pheochromocytoma over adrenal cortical carcinoma?

    1. (a)

      Calretinin

    2. (b)

      Steroidogenic Factor 1

    3. (c)

      Chromogranin A

    4. (d)

      Inhibin α

    5. (e)

      Melan A

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Sergi, C.M. (2020). Endocrine System. In: Pathology of Childhood and Adolescence. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-59169-7_11

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  • DOI: https://doi.org/10.1007/978-3-662-59169-7_11

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