In a patient with an adrenal tumor who experiences headaches that begin behind the nose and alternate between the nasal region and the right eye, what type of headache is this?

Headache Classification in Patient with Adrenal Tumor

This headache pattern most likely represents a secondary headache related to pheochromocytoma, specifically a catecholamine-induced headache, given the temporal relationship with adrenal tumor development and the characteristic unilateral periorbital pain distribution.

Primary Diagnostic Consideration: Pheochromocytoma-Related Headache

The key clinical features pointing toward pheochromocytoma include:

• Temporal relationship: Headaches began after adrenal tumor development, suggesting a secondary rather than primary headache disorder 1
• Pain location: Retronasal origin migrating to periorbital region is consistent with catecholamine-induced vascular headache patterns seen in pheochromocytoma 1
• Unilateral distribution: The strictly unilateral nature with orbital involvement matches the pain distribution seen in catecholamine excess states 1

Immediate Biochemical Evaluation Required

Before any further diagnostic workup, you must obtain plasma free metanephrines or 24-hour urinary fractionated metanephrines to confirm or exclude pheochromocytoma 2, 3. This is critical because:

• Pheochromocytomas cause headaches in the majority of patients due to catecholamine hypersecretion 1
• The headache pattern (episodic, severe, with specific location) is consistent with catecholamine surges 1
• Fine needle biopsy is absolutely contraindicated before excluding pheochromocytoma biochemically, as it can trigger fatal hypertensive crisis 1, 2, 3

Differential Diagnoses to Consider

Primary Headache Disorders (Less Likely Given Tumor History)

Cluster headache can be excluded because 4, 5:

• Cluster attacks last 15-180 minutes, not the alternating pattern described
• Cluster headache has marked autonomic features (lacrimation, nasal congestion, ptosis) during every attack 4
• Patients with cluster headache are typically restless and agitated during attacks 4
• No mention of these characteristic features in this patient

Migraine with cranial autonomic symptoms is possible but less likely 1:

• Pediatric migraineurs can have nasal symptoms (62% have cranial autonomic features) 1
• However, the temporal relationship with adrenal tumor development strongly suggests secondary etiology 1
• Migraine typically has longer attack duration and associated features like photophobia, nausea 1

Other Secondary Headache Considerations

SUNCT syndrome (Short-lasting Unilateral Neuralgiform headache with Conjunctival injection and Tearing) could mimic this pattern 5:

• Characterized by very brief attacks (seconds to minutes) with marked autonomic activation 5
• However, SUNCT is rare and would not explain the temporal relationship with adrenal tumor 5

Diagnostic Algorithm

1. Obtain plasma free metanephrines immediately (preferred test for catecholamine excess) 2, 3
2. Perform unenhanced CT of abdomen to characterize the adrenal mass (measure Hounsfield units) 6, 3
3. Complete hormonal screening including:
   • 1 mg overnight dexamethasone suppression test for cortisol excess 3, 7
   • Aldosterone-to-renin ratio if hypertension or hypokalemia present 3, 7
4. If biochemistry confirms pheochromocytoma and initial imaging is negative, extend imaging to chest and neck 2, 3
5. Consider FDG-PET for biochemically confirmed pheochromocytoma, as it is superior to MIBG for detecting malignant tumors, particularly with SDHB mutations 1, 2

Critical Management Pitfalls to Avoid

• Never perform imaging with ionic contrast agents in suspected pheochromocytoma; use nonionic agents only 2
• Never biopsy an adrenal mass before excluding pheochromocytoma due to risk of hypertensive crisis 1, 2, 3
• Do not dismiss the headache as "just migraine" when there is clear temporal relationship with adrenal tumor development 1, 3
• If pheochromocytoma is confirmed, preoperative alpha-blockade for 10-14 days is mandatory before any surgical intervention 1

Additional Considerations

If pheochromocytoma is confirmed, genetic testing should be considered, as 35% of pheochromocytomas are hereditary 1, 3. SDHB mutations in particular are associated with higher malignancy risk and may influence imaging strategy and long-term surveillance 1.