Retinal Tumors — MCQs
Which condition is characterized by the clinical features of periorbital ecchymosis (raccoon eyes) due to metastatic spread to the orbit?
Which of the following statements about cavernous hemangioma is false?
All of the following are true about the incidence of retinoblastoma except:
What is the cause of glaucoma in retinoblastoma?
A patient surgically treated for bilateral pheochromocytoma has started developing a gait abnormality. An MRI of the head was performed. What is the probable diagnosis?
Which of the following childhood tumor uses N-myc gene amplification for its prognosis?
Which melanoma subtype has the worst prognosis?
Statement 1 - A 59-year-old patient presents with flaccid bullae. Histopathology shows a suprabasal acantholytic split. Statement 2 - The row of tombstones appearance is diagnostic of Pemphigus vulgaris.
Flexner-Wintersteiner rosette is seen in-
In the context of retinal conditions, what is the primary cause of shifting fluid beneath the retina?
Retinal Tumors Indian Medical PG Practice Questions and MCQs
Question 1: Which condition is characterized by the clinical features of periorbital ecchymosis (raccoon eyes) due to metastatic spread to the orbit?
- A. Pheochromocytoma
- B. Adrenal incidentaloma
- C. Neuroblastoma (Correct Answer)
- D. Medullary thyroid cancer
Explanation: ***Neuroblastoma*** - **Periorbital ecchymosis** (raccoon eyes) is a **pathognomonic sign** of **neuroblastoma with orbital metastases** - Most common **extracranial solid tumor in children** (median age 18 months) - Originates from **neural crest cells** in the adrenal medulla or sympathetic ganglia - Orbital metastases occur via **hematogenous spread** to periorbital bones, causing hemorrhage and the characteristic "raccoon eyes" appearance - Other metastatic features: **skull bone lesions**, hepatomegaly, and bone marrow infiltration - Elevated **catecholamines** (VMA, HVA in urine) and **N-myc amplification** are key diagnostic markers *Pheochromocytoma* - Arises from **chromaffin cells** of the adrenal medulla - Presents with **episodic hypertension**, headache, palpitations, and diaphoresis due to **catecholamine excess** - Rare in children; when present, often associated with **genetic syndromes** (VHL, MEN 2, NF1) - Does **not metastasize to orbit** or cause periorbital ecchymosis - "Rule of 10s": 10% bilateral, 10% extra-adrenal, 10% malignant *Adrenal incidentaloma* - **Asymptomatic adrenal mass** discovered incidentally on imaging (CT/MRI) done for unrelated reasons - May be non-functional or hormonally active (requires biochemical workup) - Does **not present with clinical symptoms** like periorbital ecchymosis - Management depends on size, imaging characteristics, and hormonal activity *Medullary thyroid cancer* - Arises from **parafollicular C cells** of the thyroid, secreting **calcitonin** - Associated with **MEN 2A/2B** syndromes (along with pheochromocytoma) - Typically presents with **thyroid nodule**, cervical lymphadenopathy - Metastasizes to **lymph nodes, lungs, liver, and bones** but **not characteristically to orbit** - Does not cause periorbital ecchymosis
Question 2: Which of the following statements about cavernous hemangioma is false?
- A. Not associated with VHL disease
- B. Less infiltrative than capillary hemangioma
- C. Intravascular thrombosis and dystrophic calcification are seen commonly
- D. Undergo spontaneous regression (Correct Answer)
Explanation: ***Undergo spontaneous regression*** - This statement is **false** because **cavernous hemangiomas** typically do not undergo spontaneous regression. They are persistent lesions. - Spontaneous regression is more characteristic of **capillary hemangiomas** (also known as infantile hemangiomas), especially those occurring in early childhood. *Less infiltrative than capillary hemangioma* - This statement is **true**. Cavernous hemangiomas are generally **well-circumscribed** and less infiltrative than capillary hemangiomas, which can sometimes spread more diffusely. - Their distinct, large vascular channels make them easier to delineate from surrounding tissue. *Intravascular thrombosis and dystrophic calcification are seen commonly* - This statement is **true**. The slow blood flow and large, irregular vascular spaces within cavernous hemangiomas predispose them to **thrombosis**. - Subsequent organization of thrombi and **fibrin deposition** often leads to **dystrophic calcification**, which can be visualized radiographically. *Not associated with VHL disease* - This statement is **true**. **Cavernous hemangiomas** are generally **not associated with von Hippel-Lindau (VHL) disease**. - VHL disease is primarily linked to **hemangioblastomas**, particularly in the CNS and retina, which are distinct from common cavernous hemangiomas.
Question 3: All of the following are true about the incidence of retinoblastoma except:
- A. Is the most common intraocular tumour of childhood
- B. No sex predisposition
- C. More common in African children than Caucasian children (Correct Answer)
- D. Occurs in 1 in 14000-34000 live births
Explanation: ***More common in African children than Caucasian children*** - This statement is **false**, as retinoblastoma incidence shows **no significant racial predilection**. The reported incidence rates are globally consistent across different ethnic groups. - While healthcare access and outcomes may vary, the inherent genetic predisposition and occurrence rate are similar worldwide, independent of race. *Is the most common intraocular tumour of childhood* - This is a **true** statement; retinoblastoma is indeed the most frequently diagnosed primary **intraocular malignancy** in children. - It accounts for a significant proportion of childhood eye cancers, often presenting before the age of five. *No sex predisposition* - This is a **true** statement; studies consistently show that the incidence of retinoblastoma is **equal between male and female children**. - There is no statistically significant difference in the occurrence of this cancer based on sex. *Occurs in 1 in 14000-34000 live births* - This is a **true** statement; the worldwide incidence of retinoblastoma is estimated to be approximately **1 in 14,000 to 1 in 34,000 live births**. - This relatively low but consistent incidence makes it a rare but significant childhood cancer.
Question 4: What is the cause of glaucoma in retinoblastoma?
- A. Neovascularisation (Correct Answer)
- B. Mass effect of the tumour
- C. Blockage of trabecular network
- D. Lysis of the lens
Explanation: ***Neovascularisation*** - Retinoblastoma leads to **neovascularization of the iris (NVI)** and **angle structures** due to tumor necrosis, ischemia, and release of **angiogenic factors (VEGF)**. - The **neovascular membrane** grows over and obstructs the **trabecular meshwork** and anterior chamber angle, causing **secondary neovascular glaucoma**. - This is the **most characteristic and common mechanism** of glaucoma in advanced retinoblastoma. - Neovascular glaucoma in retinoblastoma is typically **refractory to medical treatment** and indicates poor prognosis. *Blockage of trabecular network* - While tumor cells can directly seed into the anterior chamber and block the trabecular meshwork, this mechanism is **less common** than neovascularization. - This option is also **less specific** as it doesn't identify the underlying pathophysiological process (neovascularization) that is characteristic of retinoblastoma-associated glaucoma. - Direct trabecular blockage by tumor cells typically occurs in advanced disease and often coexists with neovascularization. *Mass effect of the tumour* - The tumor mass itself rarely causes glaucoma through direct mechanical compression of outflow pathways. - Retinoblastoma causes glaucoma primarily through **secondary mechanisms** such as inflammation, neovascularization, or cellular seeding, not simple bulk effect. - Massive tumors may cause angle closure, but this is uncommon compared to neovascular mechanisms. *Lysis of the lens* - **Phacolytic glaucoma** from lens protein leakage is rare in retinoblastoma. - While lens damage can occur with advanced tumors, it is **not a typical or characteristic cause** of glaucoma in retinoblastoma. - The primary mechanisms involve the **tumor-angle-trabecular meshwork axis**, not lens pathology.
Question 5: A patient surgically treated for bilateral pheochromocytoma has started developing a gait abnormality. An MRI of the head was performed. What is the probable diagnosis?
- A. Multiple endocrine neoplasia type 4
- B. Von Hippel Landau Syndrome (Correct Answer)
- C. Autoimmune polyglandular syndrome
- D. Multiple endocrine neoplasia type 2B
Explanation: **Von Hippel Landau Syndrome** - This syndrome is characterized by a predisposition to developing various tumors, including **pheochromocytomas** (which are often bilateral) and central nervous system **hemangioblastomas**, which can cause gait abnormalities. - The presence of **bilateral pheochromocytoma** and the subsequent development of **gait abnormality** requiring a head MRI strongly suggests VHL, as **cerebellar hemangioblastomas** are a common feature that would explain the neurologic symptoms. *Multiple endocrine neoplasia type 4* - MEN4 involves a genetic predisposition to tumors of the **parathyroid, pituitary, and adrenal glands**, but it is less commonly associated with bilateral pheochromocytomas or specific neurological manifestations like hemangioblastomas causing gait issues. - It is a more recently described syndrome with overlapping features with other MEN disorders but does not typically include CNS tumors as a prominent feature. *Autoimmune polyglandular syndrome* - This syndrome is characterized by **autoimmune destruction of multiple endocrine glands**, leading to deficiencies (e.g., Addison's disease, type 1 diabetes), rather than tumor formation like pheochromocytomas. - It does not involve the formation of CNS tumors such as hemangioblastomas that would cause a gait abnormality. *Multiple endocrine neoplasia type 2B* - MEN2B is strongly associated with **medullary thyroid carcinoma**, **pheochromocytoma** (often bilateral), **mucosal neuromas**, and a **marfanoid habitus**. - While it includes pheochromocytomas, it does not typically involve CNS hemangioblastomas leading to gait abnormalities, which are a hallmark of VHL.
Question 6: Which of the following childhood tumor uses N-myc gene amplification for its prognosis?
- A. Neuroblastoma (Correct Answer)
- B. Nephroblastoma
- C. Retinoblastoma
- D. Rhabdomyosarcoma
Explanation: ***Neuroblastoma*** - **N-myc gene amplification** is a crucial **prognostic indicator** in neuroblastoma, correlating with aggressive disease and poor outcomes [1]. - Neuroblastoma is a **childhood cancer of neural crest origin**, often presenting as an adrenal mass or in sympathetic ganglia. *Nephroblastoma* - Also known as **Wilms tumor**, it is a childhood kidney cancer. - Its prognosis is more strongly associated with histology (e.g., **anaplasia**) and specific gene mutations like **WT1**, not N-myc amplification. *Retinoblastoma* - This is a **childhood eye cancer**. - Its prognosis is primarily linked to the presence of **RB1 gene mutations** and the extent of retinoblastoma gene protein (pRB) expression, not N-myc. *Rhabdomyosarcoma* - An aggressive **childhood soft tissue sarcoma** with skeletal muscle differentiation. - Prognostic factors often include clinical staging, histology (e.g., **alveolar vs. embryonal**), and specific genetic translocations like **PAX-FOXO1**, rather than N-myc amplification. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 486-487.
Question 7: Which melanoma subtype has the worst prognosis?
- A. Superficial spreading
- B. Nodular melanoma (Correct Answer)
- C. Lentigo maligna melanoma
- D. Acral lentiginous melanoma
Explanation: ***Nodular melanoma*** - This subtype has the **worst prognosis** among melanoma subtypes due to its aggressive growth pattern. - It exhibits **vertical growth from the outset** with no radial growth phase, leading to rapid deep invasion. - Typically presents at a more advanced stage with greater **Breslow thickness** at diagnosis. - High metastatic potential and rapid progression contribute to poorer survival rates. *Acral lentiginous melanoma* - Occurs on **palms, soles, and subungual areas** and is more common in darker-skinned populations. - Poor outcomes are primarily due to **delayed diagnosis** and late presentation rather than inherently aggressive biology. - When diagnosed at comparable stages, prognosis is similar to other melanoma subtypes. *Superficial spreading melanoma* - The **most common melanoma subtype** (60-70% of cases). - Has a prolonged **horizontal (radial) growth phase** before vertical invasion. - Generally better prognosis due to longer period allowing for **early detection and treatment**. *Lentigo maligna melanoma* - Occurs on **chronically sun-damaged skin**, typically on the face and neck of elderly patients. - Has a prolonged **in situ phase** (lentigo maligna) before invasive melanoma develops. - Generally has a **better prognosis** when detected and treated during the in situ phase.
Question 8: Statement 1 - A 59-year-old patient presents with flaccid bullae. Histopathology shows a suprabasal acantholytic split. Statement 2 - The row of tombstones appearance is diagnostic of Pemphigus vulgaris.
- A. Statements 1 & 2 are correct, 2 is not explaining 1 (Correct Answer)
- B. Statements 1 and 2 are correct and 2 is the correct explanation for 1
- C. Statements 1 and 2 are incorrect
- D. Statement 1 is incorrect
Explanation: ***Correct: Statements 1 & 2 are correct, 2 is not explaining 1*** **Analysis of Statement 1:** - A 59-year-old patient with **flaccid bullae** and **suprabasal acantholytic split** on histopathology is the classic presentation of **Pemphigus vulgaris** - The flaccid (easily ruptured) nature of bullae distinguishes it from tense bullae seen in bullous pemphigoid - The suprabasal location of the split (just above the basal layer) with acantholysis (loss of cell-to-cell adhesion) is pathognomonic - **Statement 1 is CORRECT** ✓ **Analysis of Statement 2:** - The **"row of tombstones" or "tombstone appearance"** is indeed a diagnostic histopathological feature of Pemphigus vulgaris - This appearance results from basal keratinocytes remaining attached to the basement membrane while suprabasal cells separate due to acantholysis - The intact basal cells standing upright resemble a row of tombstones - **Statement 2 is CORRECT** ✓ **Does Statement 2 explain Statement 1?** - Statement 2 describes a **histopathological appearance** (tombstone pattern) that is a **consequence** of the suprabasal split - However, it does NOT explain the **underlying cause** of the flaccid bullae or the suprabasal split - The true explanation involves **IgG autoantibodies against desmoglein 3 (and desmoglein 1)**, which attack intercellular adhesion structures (desmosomes), causing **acantholysis** - Therefore, **Statement 2 does NOT explain Statement 1** ✗ *Incorrect: Statement 2 is the correct explanation for Statement 1* - While both statements describe features of Pemphigus vulgaris, the tombstone appearance is a descriptive finding, not an explanatory mechanism *Incorrect: Statements 1 and 2 are incorrect* - Both statements are medically accurate descriptions of Pemphigus vulgaris features *Incorrect: Statement 1 is incorrect* - Statement 1 correctly describes the cardinal clinical and histopathological features of Pemphigus vulgaris
Question 9: Flexner-Wintersteiner rosette is seen in-
- A. Retinoblastoma (Correct Answer)
- B. Hepatoblastoma
- C. Nephroblastoma
- D. Neuroblastoma
Explanation: ***Retinoblastoma*** - Flexner-Wintersteiner rosettes are **characteristic histological features** seen in retinoblastoma, indicating retinal differentiation [1]. - These rosettes reflect the **presence of photoreceptor-like structures**, which are specific to this type of tumor [1]. *Hepatoblastoma* - Histologically, hepatoblastoma shows **primitive epithelial cells** and **mixed patterns**, not Flexner-Wintersteiner rosettes. - It is primarily associated with **liver** and does not present with retinal differentiation. *Nephroblastoma* - Nephroblastoma, or Wilms tumor, typically exhibits **triphasic histology** (epithelial, stromal, and blastemal components) without rosette formation. - It primarily affects the **kidney** and does not involve the retina. *Neuroblastoma* - Neuroblastoma is characterized by **small round blue cells** and **neuroid differentiation** but lacks Flexner-Wintersteiner rosettes. - This tumor usually arises in the **adrenal glands** or sympathetic nervous system, not in retinal tissue. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Eye, p. 1342.
Question 10: In the context of retinal conditions, what is the primary cause of shifting fluid beneath the retina?
- A. Exudative Retinal detachment (Correct Answer)
- B. Tractional Retinal Detachment
- C. Rhegmatogenous retinal detachment
- D. Retinodialysis
Explanation: ***Exudative Retinal detachment*** - This condition is characterized by the accumulation of **serous fluid** beneath the retina without a retinal break, causing the retina to detach. The fluid can shift with changes in head position due to gravity, leading to a **"shifting fluid" phenomenon**. - It results from conditions that compromise the **retinal pigment epithelium (RPE)** or choroidal vasculature, such as **choroidal tumors**, **inflammatory diseases**, or **severe hypertension**, leading to leakage of fluid. *Tractional Retinal Detachment* - This type of detachment occurs when **fibrovascular membranes** on the retinal surface contract and pull the neurosensory retina away from the RPE. - The detachment is usually **immobile** or minimally mobile because it is held in place by fibrous adhesions, and therefore, does not typically exhibit shifting fluid. *Rhegmatogenous retinal detachment* - This is the most common type of retinal detachment and occurs due to a **full-thickness break or tear** in the retina, allowing vitreous fluid to pass into the subretinal space. - While fluid is present, the key feature is a retinal break, and the detached retina is typically more fixed by the flow through the break rather than gravitationally shifting. *Retinodialysis* - Retinodialysis is a specific type of **rhegmatogenous retinal detachment** characterized by a **disinsertion of the retina from its ora serrata attachment**, often due to trauma. - Similar to other rhegmatogenous detachments, fluid accumulates in the subretinal space, but the primary cause is the tear/disinsertion, and it doesn't primarily manifest as a shifting fluid characteristic, which is more indicative of exudative causes.
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