Neoplasia Practice Questions

Q: Regarding anaplastic thyroid carcinoma, which of the following statements is true?

Associated with p53 mutation. ***Associated with p53 mutation*** - **Anaplastic thyroid carcinoma** is a highly aggressive cancer frequently characterized by **p53 tumor suppressor gene mutations**. - These mutations contribute to its rapid growth, dedifferentiation, and resistance to therapy. *Common in young* - **Anaplastic thyroid carcinoma (ATC)** is a rare and highly aggressive malignancy that primarily affects **older adults**, typically those over 60 years of age. - It is one of the least differentiated forms of thyroid cancer and has a poor prognosis. [1] *Surrounding neck tissue is usually free* - ATC is characterized by **rapid growth** and **aggressive local invasion**, often involving surrounding neck tissues such as the trachea, esophagus, and great vessels. [1] - This extensive local involvement contributes to symptoms like dysphagia, dyspnea, and hoarseness, and makes surgical resection difficult or impossible. *Radiotherapy cures* - **ATC is largely refractory to conventional therapies**, including surgery, radiotherapy, and chemotherapy, due to its aggressive biology and common presence of dedifferentiated cells. - While radiation therapy may be used for **palliative management** of local symptoms, it very rarely achieves a cure; the prognosis remains extremely poor, with median survival measured in months. [1] **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1101-1102.

Q: Which one of the following is an important tumor marker in the diagnosis of endodermal sinus tumor?

Alpha-fetoprotein. ***Alpha-fetoprotein*** - **Alpha-fetoprotein (AFP)** is consistently elevated in patients with **endodermal sinus tumor (yolk sac tumor)**, making it a crucial diagnostic marker. - The tumor cells produce AFP, reflecting their differentiation towards embryonic yolk sac structures. *HCG* - **Human chorionic gonadotropin (HCG)** is primarily associated with **choriocarcinoma** and other germ cell tumors like mixed germ cell tumors containing choriocarcinoma components [1],[2]. - While some germ cell tumors can co-express HCG and AFP, AFP is the dominant and most specific marker for endodermal sinus tumors. *CA-125* - **CA-125** is a widely used tumor marker for **epithelial ovarian cancer**. - It is not typically elevated in germ cell tumors such as endodermal sinus tumors. *LDH* - **Lactate dehydrogenase (LDH)** can be elevated in various cancers, including some germ cell tumors, reflecting high tumor burden and rapid cell turnover. - However, LDH is a **non-specific marker** and not as specific for endodermal sinus tumor as AFP. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 512-513. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Female Genital Tract, pp. 1035-1036.

Q: In Wilms' tumor, what leads to the emergence of resistance to chemotherapy?

Anaplastic features in tumor cells. ***Anaplasia*** - Anaplasia in Wilms' tumor is associated with **poor differentiation**, leading to increased **resistance to chemotherapy** due to altered cell growth and survival pathways. - Tumors exhibiting anaplastic features often have a more aggressive behavior, resulting in **treatment failure** and **recurrence** despite chemotherapy. *Capsular infiltration* - While capsular infiltration indicates **tumor aggressiveness**, it does not directly contribute to **chemotherapy resistance** mechanisms. - It primarily reflects **advanced stage** or local spread rather than alterations in drug response. *Nephrogenic rests* - Nephrogenic rests are **pre-existing lesions** that can predispose to Wilms' tumor [1] but are not directly tied to **chemotherapy resistance**. - They are typically changes related to **developmental anomalies** rather than mechanisms of drug resistance. *Monophasic morphology* - Monophasic morphology refers to the **uniform appearance** of tumor cells, which typically indicates less heterogeneity and does not imply **resistance to chemotherapy**. - This characteristic may affect prognosis but is not a mechanism contributing to **chemotherapy failure**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 486-490.

Q: Which of the following is NOT classified as a small round cell tumor?

Wilm's tumor. ***All of the above*** - Small round cell tumors encompass a variety of neoplasms including **retinoblastoma** and **rhabdomyosarcoma** [1]. - They are characterized by **primitive, undifferentiated cells**, making this classification broad and inclusive of all mentioned tumors. *Retinoblastoma (a small round cell tumor)* - While retinoblastoma is indeed a **small round cell tumor** [1], this option fails to recognize the broader classification of tumors in the question. - It doesn't encompass other key tumors that can also be categorized under the **small round cell tumor** umbrella. *Wilm's tumor (not a small round cell tumor)* - Wilm's tumor is classified as a **nephroblastoma**, featuring a distinct morphology that does not fit into the small round cell category. - Typically presents as a **pediatric abdominal mass** with **triphasic pathology**, unlike small round cell tumors which have a more uniform appearance. *Rhabdomyosarcoma (a small round cell tumor)* - Although rhabdomyosarcoma is considered a **small round cell tumor** [1][2], this option too does not cover all tumors classified under this term. - It features **mesenchymal differentiation** and uniform round cells with little cytoplasm [2] rather than presenting a broad view, as requested by the question. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 483-484. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, pp. 1224-1225.

Q: Radiation exposure can lead to which type of thyroid carcinoma?

Papillary carcinoma. ***Papillary carcinoma*** - Papillary thyroid carcinoma is strongly associated with **radiation exposure**, particularly during childhood [1]. - It is the most prevalent type of thyroid cancer and typically has a **good prognosis** [1]. *Lymphoma* - Thyroid lymphoma is rare and generally not linked to **radiation exposure**; it often presents as a **rapidly enlarging goiter**. - It is more commonly associated with **autoimmune thyroiditis**, not primary radiation effects. *Follicular carcinoma* - Follicular carcinoma shows a correlation with **iodine deficiency** rather than radiation exposure [1]. - Its presentation is more subtle, compared to the classical association of **radiation with papillary carcinoma**. *Medullary carcinoma* - Medullary thyroid carcinoma is primarily linked to **familial syndromes** like MEN 2 and not radiation exposure. - It arises from **parafollicular C cells**, making it clinically distinct from radiation-related types. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1098-1099.

Q: Which of the following statements about borderline tumors is false?

Metastases are common. ***Metastases are common*** - This statement is **false** because **metastases are rare** in borderline ovarian tumors [1]. - While they can have intra-abdominal implants, these are usually non-invasive and do not typically metastasize to distant sites like invasive carcinomas [2]. *10% of all epithelial tumours are borderline* - Borderline ovarian tumors, also known as **tumors of low malignant potential**, account for approximately **10-15% of all epithelial ovarian tumors**. - This percentage indicates they are a significant, but not predominant, subtype of ovarian epithelial neoplasms. *they have a good prognosis* - Patients with borderline ovarian tumors generally have an **excellent prognosis**, with a 5-year survival rate typically over 90% [1], [2]. - This is due to their non-invasive nature and low potential for aggressive spread. *Absence of stromal invasion* - A key defining characteristic of borderline ovarian tumors is the **absence of destructive stromal invasion** [2]. - They exhibit epithelial proliferation beyond that seen in benign tumors, but without invading the underlying stroma, which differentiates them from invasive carcinomas. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Female Genital Tract Disease, pp. 478-480. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Female Genital Tract, pp. 1029-1032.

Q: Which type of cancer is most commonly associated with perineural invasion in the head and neck region?

Adenoid cystic carcinoma. ***Adenoid cystic carcinoma*** - Perineural invasion is a hallmark feature of **adenoid cystic carcinoma**, particularly in the **head and neck region** [1]. - This cancer tends to infiltrate along nerve sheaths, which can lead to **pain and neurological symptoms** due to local invasion. *Adenocarcinoma* - While adenocarcinoma can exhibit invasiveness, it is not specifically characterized by **perineural invasion** as a consistent feature. - These tumors usually arise from **glandular tissues** and do not have the same propensity for nerve invasion. *Basal cell adenoma* - Basal cell adenoma is generally a **benign tumor**, which does not exhibit aggressive features like perineural invasion. - Most frequently, they present as localized masses without significant invasion into surrounding structures. *Squamous cell carcinoma* - Although squamous cell carcinoma can be aggressive, **perineural invasion** is less commonly identified compared to other malignancies like adenoid cystic carcinoma. - It primarily involves **keratinizing epithelial cells** and tends to invade adjacent tissues rather than along nerve pathways. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Head and Neck, pp. 753-755.

Q: Chordoma commonly involves which of the following locations?

Clivus and sacrum. ***Clivus and sacrum*** - Chordomas are rare malignant bone tumors derived from remnants of the **notochord**, which primarily occur at the ends of the axial skeleton. - The most common sites are the **sacrococcygeal region (lower spine)** and the **clivus (base of the skull)**, accounting for approximately 50-60% and 30-40% of cases, respectively. *Dorsal spine* - While chordomas can occur anywhere along the axial skeleton, involvement of the **dorsal (thoracic) spine** is much less common compared to the clivus and sacrum. - This region represents a smaller percentage of overall chordoma cases. *Lumbar spine* - Similar to the dorsal spine, the **lumbar spine** is an atypical location for chordomas, with a lower incidence than the sacrum and clivus. - Although it is part of the axial skeleton, it is not one of the predilection sites for these tumors. *Cervical spine* - **Cervical spine** involvement in chordomas is rare, occurring in less than 10% of cases. - Its incidence is significantly lower compared to the clival or sacrococcygeal regions.

Q: What is Askin's tumor?

Primitive neuroectodermal tumor in chest. ***Primitive neuroectodermal tumor in chest*** - Askin's tumor is a rare, highly **malignant tumor** that arises from neuroectodermal cells, typically found in the **thoracopulmonary region**. - It is classified as a type of **peripheral primitive neuroectodermal tumor (pPNET)**, sharing genetic similarities with Ewing's sarcoma. *Cholangiocarcinoma arising from bifurcation of bile duct* - This describes a **Klatskin tumor**, which is a specific type of cholangiocarcinoma that originates at the confluence of the right and left hepatic ducts. - Klatskin tumors are **biliary tract cancers** and are distinct from neuroectodermal tumors of the chest wall. *Lung adenocarcinoma involving apical region* - This description refers to a **Pancoast tumor**, which is a type of lung cancer (most often non-small cell lung cancer, including adenocarcinoma) located in the apex of the lung. - Pancoast tumors are characterized by symptoms related to invasion of surrounding structures, such as **brachial plexus** involvement. *Enterochromaffin cell tumor* - Sometimes referred to as a **carcinoid tumor**, these are neuroendocrine tumors that arise from enterochromaffin cells primarily in the gastrointestinal tract or lungs. - They produce **amines and peptides** that can cause carcinoid syndrome but are histologically and clinically distinct from Askin's tumor.

Question 1

Regarding anaplastic thyroid carcinoma, which of the following statements is true?

Accepted Answer

Associated with p53 mutation

Answer

Common in young

Answer

Radiotherapy cures

Answer

Surrounding neck tissue is usually free

Question 2

Which one of the following is an important tumor marker in the diagnosis of endodermal sinus tumor?

Accepted Answer

Alpha-fetoprotein

Answer

HCG

Answer

CA-125

Answer

LDH

Question 3

Which of the following is not a risk factor for cholangiocarcinoma?

Accepted Answer

Aflatoxin

Answer

Thorotrast

Answer

Radon

Answer

Dioxin

Question 4

In Wilms' tumor, what leads to the emergence of resistance to chemotherapy?

Accepted Answer

Anaplastic features in tumor cells

Answer

Nephrogenic rests associated with Wilms' tumor

Answer

Tumor capsular infiltration

Answer

Monophasic tumor morphology

Question 5

Which of the following is NOT classified as a small round cell tumor?

Accepted Answer

Wilm's tumor

Answer

Ewing's sarcoma

Answer

Rhabdomyosarcoma

Answer

Retinoblastoma

Question 6

Radiation exposure can lead to which type of thyroid carcinoma?

Accepted Answer

Papillary carcinoma

Answer

Lymphoma

Answer

Medullary carcinoma

Answer

Follicular carcinoma

Question 7

Which of the following statements about borderline tumors is false?

Accepted Answer

Metastases are common

Answer

Absence of stromal invasion

Answer

10% of all epithelial tumours are borderline

Answer

They have a good prognosis

Question 8

Which type of cancer is most commonly associated with perineural invasion in the head and neck region?

Accepted Answer

Adenoid cystic carcinoma

Answer

Adenocarcinoma

Answer

Basal cell adenoma

Answer

Squamous cell carcinoma

Question 9

Chordoma commonly involves which of the following locations?

Accepted Answer

Clivus and sacrum

Answer

Dorsal spine

Answer

Lumbar spine

Answer

Cervical spine

Question 10

What is Askin's tumor?

Accepted Answer

Primitive neuroectodermal tumor in chest

Answer

Cholangiocarcinoma arising from bifurcation of bile duct

Answer

Lung adenocarcinoma involving apical region

Answer

Enterochromaffin cell tumor

Neoplasia — MCQs

Neoplasia — MCQs

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