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The pathology report diagnosis is: Cranium, right temporal area, resection of intradural, extra-axial mass: Severe acute and chronic inflammation, histiocytic reaction, and proliferative fibrosis. See comment.

Comment: Among potential alternative considerations are an infectious process, or non-infectious inflammatory CNS lesions such as inflammatory pseudotumor, Rosai-Dorfman disease, plasma cell granuloma, idiopathic hypertrophic pachymeningitis, and inflammatory myofibroblastic tumor.

The clinicians discuss this and review other chart information and conclude the patient has a clinical diagnosis of Rosai-Dorfman disease.

This is a rare disorder characterized by proliferation of histiocytes.

Cavernous hemangiomas are typically described as vascular malformations in the brain. Per a search of the literature, cavernoma, cavernous hemangioma and cavernous malformation are all synonymous. There is some controversy as to whether cavernomas are vascular malformations or tumors. Cavernous hemangioma (9121/0) has been assigned a code in the ICD-O-3. The other terms are not even listed. Benign brain guidelines indicate that named tumors that have been assigned an ICD-O-3 code are reportable. Would we report a lesion that is labeled cavernous hemangioma but not one that is labeled carvernoma? Are cavernous malformations of the brain to be reported as benign brain tumors? The MP/H guidelines for benign brain tumors do not include blood vessel tumors in chart 1.

Are the following tumors reportable? If so, what is the primary site?

Example 1: Patient admitted for resection. Clinical diagnosis is left temporal cavernous hemangioma. Path diagnosis is cerebral cortex and white matter showing cavernoma.

Example 2: Patient admitted for resection with clinical diagnosis of parietal cavernous hemangioma. Path shows A-V malformation.

Example 3: Patient had T4 spinal tumor removed. Path showed cavernous angioma.

Reference: I&R 18109 and 23460

1. Meningothelial meningioma with prominent nuclear pleomorphism, infiltration into dura, calvarium, temporalis skeletal muscle.

Microscopic: Multifocal infiltration by meningothelial tumor...extensive infiltration of trabecular spaces, extension through inner and outer calvarial layers by meningioma...mitotic activity in tumor noted but below the 4 per 10 high power field threshold for diagnosis of atypical meningioma.

2. Aggressive (invasive) transitional type meningioma, neuroimaging and histology imply extensive invasive meningioma involving bone and paraspinal soft tissues. Microscopy:...invaded bone...focal EMA positivity diagnostic of invasive transitional type meningioma... tumor invades bone.

Example: MRI found an intradural, extra-axial mass at T12/L1 with possible intramedullary component. Resection of the intradural intramedullary and extramedullary spinal cord tumor found a capillary hemangioma pathologically. The microscopic description on the path report describes a tumor with extensive vascularity involving the dura.

Should we equate the statement of capillary to mean the tumor is arising in a blood vessel as we do for venous hemangioma (non-reportable per SINQ 20130001)? Or should it be reportable as C700, 9131/0 because it is described as involving the dura (intradural, intramedullary and extramedullary)?

Patient was diagnosed on imaging with enhancement along the right optic nerve intraorbital segment, displacing the optic nerve, most consistent with optic nerve sheath meningioma.

Extracranial meningiomas are rare, however SINQ 20230052 does contain an exception for reportability in a different head and neck site because it is not an intracranial location.

It is unclear if this portion of the meninges surrounding the intraorbital optic nerve is still “intracranial” and thus reportable.