Breast joint clinic ( tumor board) Nov 5th , 2016

Case presentation :  differential diagnosis of abnormal lesion in cavernous sinus  in breast cancer

38 years old female

Right breast UOQ mass

Patho: IDC, ER+, PR+, HER2- , Ki67: 7-10%

Headache after first cession of chemotherapy

Brain MRI: Abnormal signal lesion near right optic nerve

Orbit MRI: suggests metastatic involvement in the mentioned territory

Discussion:

Cavernous sinus (CS) is a venous plexus located laterally at both sides of the sella turcica and contains important vasculonervous structures. It may be the location of a wide range of pathological processes. CS is a septated venous plexus surrounded by a dural fold and located laterally to the sella turcica . Both cavernous sinuses are interconnected by a venous plexus named the coronary sinus. The internal carotid artery (ICA) runs medially through the CS surrounded by its periarterial sympathetic plexus. The abducens nerve (VI) passes laterally to the cavernous ICA, within CS. More laterally, within the dural wall, are located from superior to inferior, the ocu-lomotor nerve (III), the trochlear nerve (IV), and the first two divisions of the trigeminal nerve (V). The third division of the cranial nerve V exits the skull through the foramen ovale without passing through the CS. CS medial wall represents the lateral limit of sella turcica, and it’s nature is controversial, either fibrous or dural. MRI does not allow an accurate direct visualisation of the CS medial wall.

 

Tumors:

Pituitary adenoma: Six to 10% of pituitary adenomas invade the cavernous sinus, and represents its most frequent lesion. Macroadenomas (>1 cm) appear hypointense on T1-weighted image (wi) and hyperintense on T2-wi. They enhance after gadolinium administration, but less than normal pituitary tissue. Invasion of the cavernous sinus is secondary to the perforation of its medial wall, and is very likely if the lateral intercarotid line is crossed or if the encasement of the intracavernous ICA by the tumor is greater than or equal to 67%.

Meningioma: It arises from the arachnoid cap cells attached to the dural wall of the CS. It may contain calcifications, induces adjacent hyperostosis and enhances strongly after contrast administration. The main features distinguishing it from pituitary adenoma are the presence of a large dural base and the constriction of the intracavernous ICA lumen.

Schwannoma: Intracranial schwannomas are usually developed from sensory nerves; within the CS, their origin is mainly from cranial nerves III and V. Schwannoma is a well delineated mass which appears iso  to hypointense comparing to the gray matter on T1-wi, hyperintense on T2-wi and enhances after contrast. Multiple CS schwannomas and bilateral acoustic ones are seen  in patients with neurofibromatosis type2.

 

Malignant Peripheral Nerve Sheath Tumor: Malignant peripheral nerve sheath tumor is a high-grade sarcoma that may infiltrate the CS. Large tumor size (5 cm), ill-defined infiltrative margins, rapid growth, tumor signal intensity heterogeneity, and erosion of the skull base foramina out of proportion to tumor size suggest its underlying malignant

nature .

 

Cavernous Hemangioma: CS hemangioma is more commonly seen during the fifth decade of life in female patients. It is among the most common primary CS tumors along with schwannoma and meningioma. This tumor is formed by sinusoidal spaces with endothelial lining  that contain slow-flowing or stagnant blood. A preoperative

diagnosis is important because of its propensity to bleed at the time of resection, their diagnosis may be suggested

when they show progressive “filling in” after contrast administration.

 

Chondrosarcoma: Cavernous sinus may be invaded by a chondrosarcoma, which arises from the petroclival synchondrosis. An important feature is the presence of matrix calcifications that appears speckled and/or amorphous on CT.

Chordoma: Intracranial chordoma is a rare malignant tumor usually developed from the notochordal remnants within the clivus, close to the spheno-occipital synchondrosis and may invade the CS. On CT, bone destruction and intratumoral calcifications are often seen. Small spots of T1 hyperintensities can be noted within the tumor and are related to intra tumoral hemorrhage or mucus.

 Juvenile Angiofibroma: Juvenile angiofibroma is a highly vascular tumor that affects mostly adolescent boys. It can extend into the central skull base and to the anterior part of the CS through the foramen rotundum, vidian canal, or foramen lacerum. The tumor can invade the CS directly by erosion of the pterygoid bone.

Sphenoid Sinus Carcinoma: Malignant tumors of the sphenoid sinus include squamous cell carcinoma and adenocarcinoma. They tend to destroy bone and directly spread to the CS.

Rhabdomyosarcoma: Rhabdomyosarcoma is a mesenchymal malignant tumor occurring most often in children. Involvement of the CS is common and is a grave prognostic sign. Destruction of the skull base with intracranial and CS involvement is present in advanced cases.

Leukemia/lymphoma: They usually involve the CS by direct extension from the nasopharyngeal structures, or by hematogeneous dissemination. An infiltration of the skull base bone and an encasement without constriction of the carotid artery can be seen.

Nasopharyngeal carcinoma: It can involve the CS by direct skull base invasion, through the foramen lacerum, or along the branches V2 and V3 of trigeminal nerve.

Cavernous sinus metastasis: Metastases are infrequent and secondary to perineural or hematogeneous extension, from head and neck cancer or distant primary malignancy. CS appears enlarged by an enhanced tissular mass with bulging of its lateral walls. Metastases to the CS can be hematogenous or perineural in nature. Distant tumors with hematogenous spread to the CS are generally renal, gastric, thyroid, lung, and breast cancers.MR imaging shows CS enlargement, outward bowing of its lateral wall, and replacement of the Meckel cave with soft tissue that homogeneously enhances. Perineural tumor spread is commonly seen along branches of cranial nerve V.Perineural spread is most commonly seen with adenoid cystic or squamous cell carcinoma but may also be seen with lymphoma, melanoma, basal cell carcinoma, rhabdomyosarcoma, neurogenic tumors, and juvenile angiofibroma. MR imaging features of perineural tumor spread include nerve enlargement and enhancement and foraminal enlargement and destruction.

 

Vascular lesions:

Intracavernous aneurysm: They represent 2—5% of intracranial aneurysms. Most are associated with vascular risk factors but intracavernous aneurysms can be secondary to trauma or septic thrombophlebitis (infected aneurysms). Vascular sequences (such as time-of-flight) are commonly used to evaluate the aneurysms.

Carotid-cavernous fistula (CCF): CCF is a traumatic or spontaneous abnormal communication between the cavernous sinus and the ICA or the meningeal arterial branches of the CS. CCF can be a direct communication between cavernous ICA and the cavernous sinus(type A) and is almost always caused by a traumatism or less frequently by a ruptured ICA aneurysm. Indirect CCF(type B, C and D) are dural shunts between the CS and the meningeal branches of the cavernous ICA and/or external carotid artery. On enhanced CT, an enlarged cavernous sinus associated with a dilated superior ophthalmic vein and uni-or bilateral proptosis can be seen. On MR imaging, flow voids within the cavernous sinus and hyperintensity on arterial 3D TOF suggest the diagnosis.

Hemangioma: It’s a vascular lesion rarely located in the CS. The characteristic imaging findings are a high signal in T2-wi and a progressive and strong centripetal enhancement.

Inflammatory/infectious

Venous thrombosis: Venous thrombosis of the CS might be related to loco-regional infection spreading from sino-nasal cavities and the mastoid. Lemierre syndrome  includes an internal jugular vein (IJV) septic thrombosis caused by an oropharyngeal infection and septic metastasis. The thrombophlebitis can also be located in the cavernous sinus.

Tolosa Hunt syndrome (THS): THS is a non-specific granulomatous inflammation located in the orbital apex and extending to the cavernous sinus. THS can be bilateral in 5% of the patients. It responds strongly to systemic corticosteroid.

Tuberculous Pachymeningitis: Tuberculosis is, in some parts of the world, a relatively common cause of pachymeningitis that produces diffuse or focal extra-axial masses along the surfaces of the CS. The lesions enhance homogeneously and intensely and may be nodular.

Fungal Infection: Invasive aspergillosis may affect the sphenoid sinus in immunocompromised patients and may extend intracranially with invasion of the CS. This infection shows low signal intensity on both T1- and T2-weighted images, which is attributed to the presence of ferromagnetic elements and calcium in the fungal and mucous concretions. Rhinocerebral mucormycosis is often an acute fulminant opportunistic infection, which may affect the orbits and paranasal sinuses. Extension into the CS may result in thrombosis and thickening of the ICA walls, with narrowing of its lumen. Actinomycosis is rare and may gain access to the CS via direct extension from the ear or sinuses or by hematogenous spread. These infections should always be considered in immunosuppressed patients who present with any type of paranasal sinus disease that extends beyond the bony walls of that sinus.

Wegener Granulomatosis: Wegener granulomatosis is a rare disorder of unknown etiology associated with necrotizing granulomas in the paranasal sinuses, nasal cavities, and/or orbits. CS involvement is rare. MR imaging shows meningeal thickening and enhancement that may extend to the CS. In most patients, the diagnosis has been established before any imaging studies are obtained.

Sarcoidosis: Dural involvement can occur with sarcoidosis and extend to the CS. The presence of multiple dural-based lesions, pachymeningitis or leptomeningeal enhancement, thickened cranial nerves, thickened hypothalamus, multiple scattered brain lesions, and periventricular multifocal white matter lesions should raise the possibility of the diagnosis of sarcoidosis.

Congenital:

Epidermoid cyst: CS epidermoid cysts are rare and can involve it in three different ways; an extracavernous epidermoid cyst can invade and compress the CS. It can arise from the lateral dural wall of the CS. Finally, it can be located inside the CS. They show no enhancement.

Fat deposits: Normal fatty areas can be found within the cavernous sinus. In some specific conditions, these fat deposits can be larger as seen in patients receiving corticosteroids, in Cushing syndrome and obese people.

Dermoid cystsIt is a rare and benign congenital tumor developed from ectopic epithelial inclusions during closure of the neural tube. They contain dermal elements such as sebum, keratin, fat and hair. Dermoid cysts involving the cavernous sinus are usually developed between the two layers of its lateral dural wall and are classified as interdural. Dermoids cysts are oval and show regular limits on MRI. They present heterogeneous signal due to its heterogeneous content. They displace medially the cavernous ICA without narrowing it.

 

 References:

1-    1- Diagnostic and Interventional Imaging (2014) 95, 849—859/Imaging of the cavernous sinus lesions/A.M. Korchia, V. Cuvinciucb, J. Caetanoa,M. Beckera, K.O. Lovbladb, M.I. Vargasb

2-     2- AJNR Am J Neuroradiol 30:444 –52 _ Mar 2009 _ www.ajnr.org





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