Orbital Tumors: Secondary — MCQs

10 questions

Which condition is characterized by the clinical features of periorbital ecchymosis (raccoon eyes) due to metastatic spread to the orbit?

Most favorable prognosis after radiotherapy is in -

Which of the following conditions is least likely to cause proptosis?

A 5 year old child who presented with proptosis of one of the eyes was found to have a desmin positive tumour. What is the probable diagnosis?

What is the most common orbital tumor in children?

In carcinoma of lower lip secondaries are seen in:

Raccoon eye is a feature of:

The most common benign tumour of the orbit is

All are causes of proptosis except:

Q10

Which of the following statements about optic nerve glioma is false?

Orbital Tumors: Secondary 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: Most favorable prognosis after radiotherapy is in -

A. Teratoma
B. Desmoid
C. Seminoma (Correct Answer)
D. Melanoma

Explanation: ***Seminoma*** - **Seminoma** is highly **radiosensitive**, meaning it responds very well to radiation therapy, leading to excellent oncological outcomes. - Due to its sensitivity, even advanced seminomas can often be cured with radiotherapy, contributing to a **favorable prognosis**. *Teratoma* - **Teratomas** are generally **radioresistant**, meaning they do not respond well to radiation therapy. - Treatment for teratomas typically involves **surgical resection**, as radiation is largely ineffective. *Desmoid* - **Desmoid tumors** (aggressive fibromatosis) are locally aggressive but rarely metastasize, and their response to radiotherapy is variable. - While radiation can be used for local control, the prognosis is often complicated by **local recurrence** and challenging surgical margins. *Melanoma* - **Melanoma** is notoriously **radioresistant**, making radiation therapy a less effective primary treatment option. - It is often used for **palliative care** or in cases of local recurrence, but rarely leads to a cure or favorable prognosis when used alone.

Question 3: Which of the following conditions is least likely to cause proptosis?

A. Sarcoidosis
B. Myxoedema (Correct Answer)
C. Grave's disease
D. Pituitary adenoma

Explanation: ***Myxoedema*** - **Myxoedema** is associated with severe **hypothyroidism** and is characterized by non-pitting edema due to the accumulation of **glycosaminoglycans** in tissues. - While it can cause facial puffiness, it is **least likely** to cause **proptosis** as there is no direct mechanism for an increase in orbital contents to push the eye forward. *Grave's disease* - **Grave's disease** is the most common cause of **proptosis** due to **immune-mediated inflammation** and accumulation of **glycosaminoglycans** and fat in the orbital tissues, leading to expansion. - This condition specifically affects the **extraocular muscles** and fat, causing bulging of the eyes. *Sarcoidosis* - **Orbital sarcoidosis** can cause **proptosis** due to the formation of **granulomas** within the orbit, leading to mass effect and inflammation. - It can affect any part of the eye and orbit, leading to varied clinical presentations including axial or non-axial globe displacement. *Pituitary adenoma* - A **pituitary adenoma** itself does not directly cause proptosis as it is located in the **sella turcica**, posterior to the orbits. - However, **large adenomas** can cause proptosis indirectly if they invade the **cavernous sinus** and obstruct venous return from the orbit, or if they are associated with **acromegaly**, which can lead to bony overgrowth of the orbital structures and surrounding tissues.

Question 4: A 5 year old child who presented with proptosis of one of the eyes was found to have a desmin positive tumour. What is the probable diagnosis?

A. Neuroblastoma
B. Retinoblastoma
C. Rhabdomyosarcoma (Correct Answer)
D. Ewing's sarcoma

Explanation: ***Rhabdomyosarcoma*** - **Desmin positivity** is a characteristic immunohistochemical feature of **rhabdomyosarcoma**, as desmin is an intermediate filament found in muscle cells [1]. - In a 5-year-old child presenting with **proptosis**, rhabdomyosarcoma of the orbit is a highly probable diagnosis, as it is the most common primary malignant orbital tumor in childhood [3]. *Neuroblastoma* - Neuroblastoma is typically a tumor of neural crest origin, with classic immunohistochemical markers being **neuron-specific enolase (NSE)** and **chromogranin**, not desmin [2]. - While it can manifest with orbital metastases leading to proptosis, the desmin positivity rules it out as the primary diagnosis [3]. *Retinoblastoma* - Retinoblastoma is a malignant tumor of the retina, presenting with **leukocoria** (white pupillary reflex) and occasionally proptosis in advanced stages [4]. - It arises from neuroectodermal cells, and its characteristic markers include **synaptophysin** and **neuron-specific enolase (NSE)**, not desmin [4]. *Ewing's sarcoma* - Ewing's sarcoma is a primary malignant small round blue cell tumor of bone and soft tissue, typically marked by expression of **CD99** and a characteristic **t(11;22) translocation**. - While it can occur in the orbit, it is not desmin positive, making rhabdomyosarcoma a more likely diagnosis given the immunohistochemical findings. **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] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. (Basic Pathology) introduces the student to key general principles of pathology, both as a medical science and as a clinical activity with a vital role in patient care. Part 2 (Disease Mechanisms) provides fundamental knowledge about the cellular and molecular processes involved in diseases, providing the rationale for their treatment. Part 3 (Systematic Pathology) deals in detail with specific diseases, with emphasis on the clinically important aspects., pp. 211-212. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1323-1324. [4] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Manifestations Of Central And Peripheral Nervous System Disease, pp. 737-738.

Question 5: What is the most common orbital tumor in children?

A. Nerve sheath tumor
B. Hemangioma (Correct Answer)
C. Lymphoma
D. Meningioma

Explanation: ***Hemangioma*** - **Capillary hemangioma** is the **most common benign orbital tumor/mass** in children, typically presenting in the first few months of life. - It is characterized by **rapid growth during the first year**, followed by **spontaneous involution** (usually complete by age 5-7 years). - These lesions are composed of rapidly proliferating endothelial cells and can cause **proptosis, ptosis**, and, if large, **amblyopia** due to visual axis obstruction or induced astigmatism. - Management is often conservative (observation) unless vision-threatening, in which case systemic steroids or propranolol may be used. *Nerve sheath tumor* - **Optic nerve sheath meningiomas** and **schwannomas** are rare in children, typically presenting in older adults. - While they can cause visual impairment and proptosis, their incidence in the pediatric population is significantly lower than hemangiomas. *Lymphoma* - **Orbital lymphoma** is exceedingly rare in children and is typically a tumor of adulthood, often associated with systemic lymphoma. - When it does occur in children, it might be a manifestation of a more widespread lymphoproliferative disorder. *Meningioma* - **Meningiomas** generally arise from arachnoid cap cells and are less common in children than in adults. - In children, they are more often associated with **neurofibromatosis type 2** and tend to be more aggressive.

Question 6: In carcinoma of lower lip secondaries are seen in:

A. Preauricular LN
B. Supraclavicular LN
C. Submandibular LN (Correct Answer)
D. Mediastinal LN

Explanation: ***Submandibular LN*** - The **lower lip** drains primarily into the **submental** and **submandibular lymph nodes**. - Therefore, **metastasis** from lower lip carcinoma is most commonly found in the submandibular lymph nodes. *Preauricular LN* - **Preauricular lymph nodes** typically drain the **temporal region**, **forehead**, and sometimes the **outer ear**. - They are not the primary drainage site for the lower lip. *Supraclavicular LN* - **Supraclavicular lymph nodes** receive drainage from the neck, upper chest, and sometimes abdominal or pelvic malignancies. - While possible in advanced cases, they are not the initial or most common site for metastasis from lower lip carcinoma. *Mediastinal LN* - **Mediastinal lymph nodes** are located in the chest and primarily drain the lungs, esophagus, and other thoracic organs. - Metastasis to these nodes from a lower lip carcinoma would indicate very advanced disease and is not a common primary site.

Question 7: Raccoon eye is a feature of:

A. Le Fort 3
B. Le Fort 1
C. Both Le Fort 2 and Le Fort 3 (Correct Answer)
D. Le Fort 2

Explanation: ***Both Le Fort 2 and Le Fort 3*** - **Raccoon eyes** (bilateral periorbital ecchymosis) is a hallmark sign of **midface fractures** that involve the base of the skull and orbital regions. - **Le Fort II fractures** (pyramidal fractures) involve the nasal bones, medial orbital walls, and infraorbital rims, with the fracture line extending through the ethmoid and lacrimal bones, allowing blood to extravasate into the periorbital area. - **Le Fort III fractures** (craniofacial dysjunction) cause complete separation of the midface from the skull base, resulting in extensive trauma that commonly produces raccoon eyes. - **Both fracture types** can cause this sign, making this the most complete answer. *Le Fort 3* - While Le Fort III fractures certainly cause **raccoon eyes** due to craniofacial dysjunction and skull base involvement, this option is **incomplete**. - Selecting only Le Fort III misses the fact that **Le Fort II fractures also cause raccoon eyes**, making "both" the better answer. *Le Fort 1* - **Le Fort I fractures** are horizontal fractures of the maxilla involving the palate and lower maxillary walls ("floating palate"). - These fractures **do not extend superiorly** to involve the orbital region or skull base, so they typically **do not cause raccoon eyes**. *Le Fort 2* - While Le Fort II fractures do cause **raccoon eyes** through involvement of the medial orbital walls, ethmoid, and lacrimal bones, this option is **incomplete**. - Selecting only Le Fort II misses the fact that **Le Fort III fractures also cause raccoon eyes**, making "both" the better answer.

Question 8: The most common benign tumour of the orbit is

A. Optic nerve glioma
B. Meningioma
C. Benign-mixed tumour
D. Haemangioma (Correct Answer)

Explanation: ***Haemangioma*** - **Cavernous haemangiomas** are the most frequently encountered benign tumors of the orbit in adults. - They are typically well-circumscribed, slow-growing vascular malformations that can cause **proptosis** and visual disturbances. *Optic nerve glioma* - While optic nerve gliomas are a benign tumor, they are less common than cavernous haemangiomas in the general orbital pathology. - These tumors specifically arise from the **optic nerve** and are more prevalent in children with **neurofibromatosis type 1 (NF1)**. *Meningioma* - **Orbital meningiomas** originate from the meninges surrounding the optic nerve or within the cranial cavity, extending into the orbit. - They are considered less common than haemangiomas and often present with a slower progression of symptoms like **proptosis** and **vision loss**. *Benign-mixed tumour* - This term usually refers to a **pleomorphic adenoma of the lacrimal gland**, which is the most common epithelial tumor of the lacrimal gland, but not the overall most common benign orbital tumor. - While benign, these tumors have a potential for malignant transformation and present with distinct symptoms related to the **lacrimal gland**.

Question 9: All are causes of proptosis except:

A. Orbital cellulitis
B. Orbital tumor
C. Retinal detachment (Correct Answer)
D. Graves' disease

Explanation: ***Retinal detachment*** - **Retinal detachment** is a condition where the retina separates from the underlying supportive tissue and does not cause proptosis. - Its primary symptoms include **flashes of light**, **floaters**, and a **curtain-like shadow** in the visual field. *Orbital cellulitis* - **Orbital cellulitis** is an infection of the fat and muscles around the eye, leading to inflammation and swelling. - This swelling can push the eye forward, causing **proptosis**. *Orbital tumor* - An **orbital tumor** is a mass growing within the orbit (eye socket), which occupies space and displaces the eyeball. - This displacement typically results in **proptosis**, often unilateral and progressive. *Graves' disease* - **Graves' disease** (or Graves' ophthalmopathy) involves inflammation and swelling of the extraocular muscles and orbital fat due to autoimmune processes. - This increased volume within the orbit directly causes **proptosis** and is often bilateral.

Question 10: Which of the following statements about optic nerve glioma is false?

A. Can cause bilateral proptosis
B. Most common primary orbital tumour among children (Correct Answer)
C. Is associated with neurofibromatosis type 1
D. Typically presents with unilateral axial proptosis

Explanation: ***Most common primary orbital tumour among children*** - This statement is **FALSE** - optic nerve glioma is the most common **optic nerve tumor** in children, but NOT the most common **primary orbital tumor**. - **Capillary hemangioma** is the most common primary orbital tumor in the pediatric age group. - This is an important distinction: optic nerve gliomas account for 1-6% of all orbital tumors in children. *Can cause bilateral proptosis* - This is TRUE - optic nerve gliomas can cause bilateral proptosis, particularly in patients with **neurofibromatosis type 1 (NF1)**. - Bilateral involvement occurs in **10-15% of cases**, especially when there is chiasmal involvement extending to both optic nerves. - While unilateral presentation is more common, bilateral disease is well-recognized. *Is associated with neurofibromatosis type 1* - This is TRUE - **15-20% of patients with NF1** develop optic pathway gliomas during childhood. - Conversely, **25-50% of children with optic nerve gliomas** have underlying NF1. - This strong association makes NF1 screening essential in children diagnosed with optic nerve gliomas. *Typically presents with unilateral axial proptosis* - This is TRUE - the classic presentation is **slowly progressive unilateral axial proptosis**. - The tumor grows within the optic nerve sheath, pushing the eye forward in an axial direction. - Associated findings include visual loss, optic disc swelling, and optociliary shunt vessels.

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