Rhabdomyosarcoma — MCQs
The most common type of rhabdomyosarcoma is
Grape-like, polypoid, bulky mass protruding through vagina in 4-year old girl is characteristic of
What is the most common site of rhabdomyosarcoma in children?
RPLND and Chemotherapy may be used in management of?
Best treatment strategy for carcinoma of the anal canal:
Histology of Myositis ossificans most closely mimics which of the following?
Which of the following statements are correct regarding the management of gastrointestinal bleeding in children? I. Somatostatin or octreotide infusion should be given for at least 7 days after stoppage of initial bleeding to prevent rebleeding II. Endoscopic Sclerotherapy (EST) involves endoscopic injection of N -butyl-2-cyanoacrylate or iso-butyl-2-cyanoacrylate III. EST has upto 90 % efficacy in controlling acute bleeding IV. Following an episode of acute variceal bleeding, all patients should receive secondary prophylaxis to prevent rebleeding Select the answer using the code given below :
Ear lobe creases in a child are commonly associated with which disorder that also presents with macroglossia and macrosomia?
What is the most common malignant neoplasm of infancy?
A 4-year-old child presented with a palpable, painless, and slowly increasing abdominal mass in the right flank region, accompanied by episodes of fever and hematuria. Examination revealed hypertension. Following a CT scan of the abdomen, the patient underwent surgical resection of the mass. The gross specimen and histopathological examination are provided. Which of the following drugs is NOT approved for the management of this condition?
Rhabdomyosarcoma Indian Medical PG Practice Questions and MCQs
Question 1: The most common type of rhabdomyosarcoma is
- A. Embryonal (Correct Answer)
- B. Alveolar
- C. Pleomorphic
- D. Spindle cell
Explanation: ***Embryonal*** - The most common type of **rhabdomyosarcoma**, primarily affecting **children**, presenting with symptoms based on its site of origin. - Typically associated with a **better prognosis** compared to other types [1], commonly found in the **head and neck**, urinary tract, or retroperitoneum. *Inflammatory* - Inflammatory rhabdomyosarcoma is a rare variant and not considered common, often involving **inflammatory reactions** rather than pure sarcoma features. - This type generally does not match the typical presentation or histology associated with **rhabdomyosarcoma**. *Pleomorphic* - Pleomorphic rhabdomyosarcoma is characterized by a **diverse morphological appearance**, typically observed in adults, making it less common in the pediatric population. - This variant is associated with a **poor prognosis** [1] and is rarely the most common type in the demographic affected. *Alveolar* - Alveolar rhabdomyosarcoma usually presents in adolescents [2] and is characterized by **alveolar structures**; it is not the most prevalent type among rhabdomyosarcomas. - This type is associated with a more aggressive behavior and poorer outcomes, distinguishing it from the more common embryonal form. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, pp. 1224-1225. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Bones, Joints, and Soft Tissue Tumors, p. 1222.
Question 2: Grape-like, polypoid, bulky mass protruding through vagina in 4-year old girl is characteristic of
- A. Leiomyosarcoma
- B. Inflammatory polyp
- C. Sarcoma botryoides (Correct Answer)
- D. Fibrosarcoma
Explanation: ***Sarcoma botryoides*** - This is a highly characteristic presentation of **embryonal rhabdomyosarcoma (sarcoma botryoides)** in young girls, where the tumor forms a **grape-like (botryoid) mass** protruding from the vagina [1]. - It typically arises from the **vagina wall or cervix** in infants and young children, often presenting with bleeding or a mass [1]. *Leiomyosarcoma* - Leiomyosarcoma is a malignant tumor of **smooth muscle**, occurring more commonly in older women [2]. - It typically presents as a **solitary, firm mass** in the uterus or vagina, not typically with a grape-like appearance [2]. *Inflammatory polyp* - An inflammatory polyp is a **benign, non-malignant outgrowth** of tissue, often associated with chronic inflammation. - While it can protrude, it lacks the **destructive, bulky, and invasive nature** implied by "grape-like, polypoid, bulky mass" in the context of a child. *Fibrosarcoma* - Fibrosarcoma is a malignant tumor of **fibrous connective tissue**, which can occur anywhere in the body. - It is uncommon in the vagina, especially in young children, and generally presents as a **firm, infiltrative mass** rather than the distinctive grape-like pattern. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Female Genital Tract, pp. 1004-1005. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Female Genital Tract, pp. 1024-1025.
Question 3: What is the most common site of rhabdomyosarcoma in children?
- A. Extremities
- B. Genitourinary area
- C. Trunk
- D. Head and neck region (Correct Answer)
Explanation: ***Head and neck region*** - The **head and neck region** is the most common site for rhabdomyosarcoma in children, accounting for approximately **35-40% of cases**. - Within the head and neck, common sub-sites include the **orbit**, **parameningeal sites** (nasopharynx, paranasal sinuses, middle ear), and other **non-parameningeal sites**. *Extremities* - Rhabdomyosarcoma can occur in the **extremities**, but it is less common than in the head and neck, typically accounting for about **20% of cases**. - When present in the extremities, it often involves the **proximal portions** of the limbs. *Genitourinary area* - The **genitourinary (GU) tract** is a significant site for rhabdomyosarcoma, particularly the **bladder** and **prostate**, accounting for approximately **20% of cases**. - While common, it is not the *most* common overall site compared to the head and neck. *Trunk* - Rhabdomyosarcoma in the **trunk** (including the chest wall, abdomen, and retroperitoneum) is less frequent, accounting for about **10-15% of cases**. - These tumors can arise from various muscle groups within the trunk, but are collectively less common than head and neck involvement.
Question 4: RPLND and Chemotherapy may be used in management of?
- A. Non-seminomatous germ cell tumors of the testis (Correct Answer)
- B. Non-germ cell tumors
- C. Seminomatous germ cell tumors
- D. Lymphoma of the testis
Explanation: ***Non-seminomatous germ cell tumors of the testis*** - **Retroperitoneal lymph node dissection (RPLND)** and **chemotherapy** are key components in the management of non-seminomatous germ cell tumors (NSGCTs), especially for metastatic disease or after initial orchidectomy. - The combination therapy addresses both local nodal involvement (RPLND) and widespread micrometastases (chemotherapy), which are common in NSGCTs. *Non-germ cell tumors* - This is a broad category, and while some non-germ cell testicular tumors may require surgery or chemotherapy, **RPLND** is not a standard part of their management in the same way it is for germ cell tumors. - The specific treatment depends on the tumor type (e.g., Leydig cell tumor, Sertoli cell tumor), stage, and histology, and often involves less aggressive approaches. *Seminomatous germ cell tumors* - **Seminomas** are highly radiosensitive and often respond well to **radiation therapy**, particularly for localized disease or retroperitoneal nodal involvement. - While chemotherapy is used for metastatic seminoma, **RPLND** is generally not indicated for seminomas due to their radiosensitivity and different metastatic patterns compared to NSGCTs. *Lymphoma of the testis* - Testicular lymphoma is a type of **non-Hodgkin lymphoma** and is primarily managed with systemic **chemotherapy** (e.g., R-CHOP) and sometimes radiation therapy. - **RPLND** is not a standard treatment modality for testicular lymphoma, as it is a systemic disease requiring systemic treatment, not local surgical excision of retroperitoneal nodes.
Question 5: Best treatment strategy for carcinoma of the anal canal:
- A. Chemoradiation (Correct Answer)
- B. Radiation
- C. Surgery
- D. Chemotherapy
Explanation: ***Chemoradiation*** - Carcinoma of the anal canal is primarily treated with **chemoradiation** (combinations of chemotherapy and radiation therapy) as the standard of care to achieve **organ preservation**. - This combined approach improves local control and survival rates compared to either modality alone, making it the **primary curative strategy** for most localized anal canal cancers. *Radiation* - While radiation therapy is a crucial component of anal canal cancer treatment, using it alone (**monotherapy**) is generally less effective than chemoradiation. - **Local recurrence rates** are higher with radiation alone compared to combined modality treatment. *Surgery* - Surgery, specifically **abdominoperineal resection (APR)**, is typically reserved for **recurrent disease** or cases where chemoradiation fails. - Initial radical surgery for anal canal cancer leads to significant morbidity (e.g., permanent colostomy) and is generally avoided as a primary treatment due to the success of chemoradiation. *Chemotherapy* - Chemotherapy alone is **not curative** for localized anal canal carcinoma. - It is primarily used in combination with radiation (chemoradiation) to sensitize the tumor to radiation and improve local control, or as treatment for **metastatic disease**.
Question 6: Histology of Myositis ossificans most closely mimics which of the following?
- A. Giant Cell Tumor
- B. Ewing's tumor
- C. Osteochondroma
- D. Osteosarcoma (Correct Answer)
Explanation: ***Osteosarcoma*** - Histologically, myositis ossificans can mimic **osteosarcoma**, as both conditions show features of **ossification and bone formation** [1]. - **Age** and **location** may also help differentiate, but careful histological examination reveals similarities. *Osteochondroma* - Characterized by a **cartilaginous cap** overlying the bone, which is not present in myositis ossificans [2]. - Usually develops in patients with a **pedunculated or sessile base**, rather than the heterogeneous ossification seen in myositis ossificans. *Ewing's tumour* - This is a **malignant round cell tumor** that predominantly affects children and young adults, with specific **translocation** genetic features. - Histologically, it shows a **small, blue cell appearance**, distinct from the fibroblastic reaction and bone formation seen in myositis ossificans. *GCT* - Granulosa cell tumors (GCT) are primarily **gynecological**, with no significant **bony changes** as seen in myositis ossificans. - Histologically, GCTs show **multinucleated giant cells** and are associated with **subarticular bone lesions**, unlike the heterotopic ossification seen in myositis ossificans. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Osteoarticular And Connective Tissue Disease, pp. 673-674. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Osteoarticular And Connective Tissue Disease, pp. 672-673.
Question 7: Which of the following statements are correct regarding the management of gastrointestinal bleeding in children? I. Somatostatin or octreotide infusion should be given for at least 7 days after stoppage of initial bleeding to prevent rebleeding II. Endoscopic Sclerotherapy (EST) involves endoscopic injection of N -butyl-2-cyanoacrylate or iso-butyl-2-cyanoacrylate III. EST has upto 90 % efficacy in controlling acute bleeding IV. Following an episode of acute variceal bleeding, all patients should receive secondary prophylaxis to prevent rebleeding Select the answer using the code given below :
- A. III and IV (Correct Answer)
- B. I and III
- C. II and IV
- D. I and II
Explanation: ***III and IV*** **Statement I is INCORRECT:** Somatostatin or octreotide infusions are typically administered for **3-5 days** (not 7 days) after initial bleeding has stopped to prevent rebleeding. Prolonged use beyond this period is not generally recommended due to potential side effects and lack of additional benefit. **Statement II is INCORRECT:** Endoscopic Sclerotherapy (EST) for esophageal varices involves injection of sclerosants such as **ethanolamine oleate, sodium tetradecyl sulfate**, or polidocanol. The tissue adhesives **N-butyl-2-cyanoacrylate** or **iso-butyl-2-cyanoacrylate** are used in **endoscopic variceal obturation (EVO)** for **gastric varices**, not in standard sclerotherapy for esophageal varices. **Statement III is CORRECT:** Endoscopic Sclerotherapy (EST) demonstrates **up to 90% efficacy** in controlling acute variceal bleeding, making it a highly effective intervention for managing acute hemorrhage. **Statement IV is CORRECT:** Following an episode of acute variceal bleeding, **all patients should receive secondary prophylaxis** (typically combination of non-selective beta-blockers and endoscopic band ligation) to prevent rebleeding, which is associated with significant morbidity and mortality. *I and III* - Incorrect because Statement I is wrong about the duration of vasoactive drug therapy. *II and IV* - Incorrect because Statement II confuses sclerotherapy agents with tissue adhesives used for gastric varices. *I and II* - Incorrect because both statements I and II contain inaccuracies regarding duration of therapy and sclerosing agents respectively.
Question 8: Ear lobe creases in a child are commonly associated with which disorder that also presents with macroglossia and macrosomia?
- A. Down syndrome
- B. Beckwith-Wiedemann syndrome (Correct Answer)
- C. Turner syndrome
- D. Noonan syndrome
Explanation: ***Beckwith-Wiedemann syndrome*** - **Beckwith-Wiedemann syndrome** is characterized by conditions such as **macrosomia** (large body size), **macroglossia** (enlarged tongue), **visceromegaly**, and **ear lobe creases** or pits. - It is an overgrowth disorder often associated with an increased risk of certain childhood cancers like **Wilms tumor** and hepatoblastoma. *Down syndrome* - **Down syndrome** (Trisomy 21) presents with distinct facial features like a **flat nasal bridge**, **epicanthal folds**, and a single palmar crease, but **ear lobe creases** are not a primary characteristic. - While **macroglossia** can be seen, **macrosomia** is generally not a feature; instead, individuals with Down syndrome often have growth delays. *Turner syndrome* - **Turner syndrome** (XO karyotype) is characterized by features such as **short stature**, a **webbed neck**, shield chest, and **low-set ears**, but not typically ear lobe creases or macroglossia. - Affected individuals are phenotypically female and experience **gonadal dysgenesis**. *Noonan syndrome* - **Noonan syndrome** shares some features with Turner syndrome, including **short stature**, **webbed neck**, and **pectus excavatum**, but also presents with distinct cardiac defects. - While it can involve various facial dysmorphia, **ear lobe creases**, **macroglossia**, and **macrosomia** are not typically defining characteristics.
Question 9: What is the most common malignant neoplasm of infancy?
- A. Malignant teratoma
- B. Neuroblastoma (Correct Answer)
- C. Wilms' tumor
- D. Hepatoblastoma
Explanation: **Explanation:** **Neuroblastoma** is the most common extracranial solid tumor of childhood and the **most common malignant neoplasm of infancy** (defined as children <1 year of age). It originates from primordial neural crest cells of the sympathetic nervous system, most commonly occurring in the adrenal medulla. Its high incidence in infancy is attributed to its embryonal nature; in fact, many cases are congenital or detected during prenatal screening. **Analysis of Incorrect Options:** * **Malignant Teratoma:** While teratomas are the most common germ cell tumors in neonates (specifically Sacrococcygeal teratoma), the majority are benign at birth. They do not surpass neuroblastoma in overall malignant frequency during infancy. * **Wilms’ Tumor (Nephroblastoma):** This is the most common primary renal tumor in children, but its peak incidence occurs between **2 to 5 years** of age. It is relatively rare in the first year of life. * **Hepatoblastoma:** This is the most common liver malignancy in children, but it is significantly less common overall than neuroblastoma. **High-Yield Clinical Pearls for NEET-PG:** * **Median age of diagnosis:** 19 months (but it remains the #1 malignancy in the <1-year age group). * **Clinical Feature:** Often presents as a hard, irregular abdominal mass that **crosses the midline** (unlike Wilms’ tumor, which usually does not). * **Opsoclonus-Myoclonus Syndrome:** A classic paraneoplastic syndrome associated with neuroblastoma ("dancing eyes, dancing feet"). * **Biomarker:** Elevated urinary catecholamines (VMA and HVA) are found in 90% of cases. * **Prognosis:** Age is a major prognostic factor; infants (<18 months) generally have a much better prognosis, including the possibility of spontaneous regression (Stage 4S).
Question 10: A 4-year-old child presented with a palpable, painless, and slowly increasing abdominal mass in the right flank region, accompanied by episodes of fever and hematuria. Examination revealed hypertension. Following a CT scan of the abdomen, the patient underwent surgical resection of the mass. The gross specimen and histopathological examination are provided. Which of the following drugs is NOT approved for the management of this condition?
- A. Doxorubicin
- B. Dactinomycin
- C. Vincristine
- D. Bleomycin (Correct Answer)
Explanation: ***Bleomycin*** - **Bleomycin** is NOT part of standard **Wilms' tumor** chemotherapy protocols (NWTS/COG regimens). - It is primarily used for **germ cell tumors** and **lymphomas**, not for **nephroblastoma**. *Doxorubicin* - **Doxorubicin** is a key component of **intermediate** and **high-risk** Wilms' tumor treatment protocols. - It is used in combination with other agents for **stage III-V** disease or **unfavorable histology**. *Dactinomycin* - **Dactinomycin** is a cornerstone drug in **all risk groups** of Wilms' tumor treatment. - It is part of the **standard two-drug regimen** along with vincristine for **favorable histology** cases. *Vincristine* - **Vincristine** is universally used in **all Wilms' tumor protocols** regardless of stage or histology. - It forms the **backbone** of treatment, often combined with dactinomycin in **low-risk** cases.
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