The sellar and parasellar (SPS) region is a complex area rich in vital neurovascular structures and as such may be the location of first manifestation of a systemic malignancy. Metastases to this region are rare; breast cancer is the most common source among those that metastasize to the SPS region. Ophthalmoplegia, headache, retroorbital or facial pain, diabetes insipidus, and visual field defects are the most commonly reported symptoms. Lack of specific clinical and radiological features renders SPS metastases difficult to differentiate from the other frequently encountered lesions in this area, especially when there is no known history of a primary disease. Currently accepted management is multimodality therapy that includes biopsy and/or palliative surgical resection, radiation, and chemotherapy. Although no significant survival benefits have been shown by the surgical series, surgical resection may improve quality of life. Here we review the relevant literature and present six illustrative cases from our own institution.
Metastatic lesions comprise approximately 1% of the tumors in the sellar/parasellar (SPS) area for which patients undergo transsphenoidal surgery (TSS) [
Despite the advancement in the imaging modalities, tumors that have metastasized to the SPS areas may still be difficult to differentiate from pituitary adenoma on radiographic studies [
Management options are multimodal and vary depending on whether a primary source is known or on the likely differential diagnoses based on the clinical and radiological findings. Multimodal options include radiation therapy, chemotherapy, and/or surgery [
In this paper, we review the clinical, endocrine, and radiological features of the metastatic SPS tumors with currently accepted therapeutic options based on the pertinent literature. In addition, we report six cases from our institution and discuss their management with long-term clinical outcome.
A systematic review of the literature was performed using PubMed and the bibliographies of reviewed articles. The medical records of six patients admitted to the University of Utah Health Sciences Center between 2001 and 2011 were reviewed retrospectively. Clinical presentation, radiographic studies, treatment, histopathological confirmation, outcome, and prognosis were recorded (Table
Clinical, endocrine, and radiological features of 6 patients with metastatic tumors of the sellar/parasellar region.
Patient | Age | Sex | Presenting symptoms | Symptom duration | Primary disease | Metastatic lesion | Location | Management | Outcome |
---|---|---|---|---|---|---|---|---|---|
1 | 77 | m | Retroorbital pain | 4 months | Prostate cancer | Prostate cancer | CS and sella extending to inferior orbital fissure and upper clivus | Transsphenoidal biopsy, radiotherapy, chemotherapy | Stable neurological findings at two months |
2 | 82 | f | Hearing loss | Several weeks | Breast cancer | Breast cancer | Petrous apex extending to Meckel’s cave, lateral CS, and IAC | Transcranial biopsy, radiotherapy, chemotherapy | N/A |
3* | 79 | m | Cranial nerve III and VI palsies | Several weeks | Prostate cancer | Melanoma | Bilateral CS, sella, clivus, and posterior nasal cavity | Transsphenoidal biopsy | N/A |
4 | 21 | m | Horner's syndrome | 1 month | Osteosarcoma | Renal cell cancer | CS extending to sphenoid sinus, pterygopalatine fossa, and optic canal | Radiation, chemotherapy | N/A |
5 | 42 | f | Gait instability | 1 month | Unknown | Lymphoma | Sellar/suprasellar lesion with bilateral CS, medial sphenoid wing, and clivus involvement | Transsphenoidal biopsy, chemotherapy, possible radiotherapy | N/A |
6 | 57 | f | Decreased visual acuity | 1 month | Breast cancer | Breast cancer | Greater sphenoid wing extending to the anterior clinoidal process with the optic nerve encasement | Transcranial decompression of optic nerve and the CS, radiotherapy with possible chemotherapy | Stable neurological findings and primary disease at one year |
CS: cavernous sinus, IAC: internal acoustic canal, N/A: not available
*Patient was previously presented in McCutcheon et al.
A 77-year-old man with known prostate cancer presented with a four-month history of left retro-orbital pain followed by left eye ptosis. At presentation, he had complete left third nerve palsy. His visual acuity was intact in both eyes, with full visual fields to confrontation. Brain magnetic resonance (MR) imaging (Figure
Axial and coronal T1-weighted MR imaging of the brain with gadolinium enhancement showing a heterogeneously enhancing mass involving the sella with invasion into the left cavernous sinus and the superior clivus.
An 82-year-old woman with known history of breast cancer presented with several weeks’ complaint of progressive left-sided hearing loss as well as facial pain and numbness in the first and the second divisions of the trigeminal nerve, respectively. On neurological examination, left-sided hearing loss and facial numbness along the V1 and V2 distributions were confirmed. MR imaging showed evidence of a heterogeneously enhancing mass in the left petrous apex that extended to involve Meckel’s cave, the lateral cavernous sinus, and the internal auditory canal (Figure
Axial and coronal T1-weighted MR imaging of the brain with gadolinium enhancement demonstrating a heterogeneously enhancing left petrous apex mass with extension into the adjacent middle cranial fossa and cerebellar pontine angle.
A 79-year-old man with known history of prostate cancer presented with several weeks’ history of progressively worsening double vision and eventual right eye ptosis. Neurological examination revealed complete third and sixth nerve palsies on the right side. The visual fields were full to confrontation in both eyes with intact visual acuity. MR imaging disclosed an enhancing soft tissue mass involving the clivus, pituitary fossa, cavernous sinus, and posterior nasal cavity (Figure
Axial and coronal T1-weighted MR imaging with gadolinium enhancement showing a homogenously enhancing soft tissue mass involving the clivus, pituitary fossa, cavernous sinus, and posterior nasal cavity.
A 21-year-old man with a remote history of osteosarcoma and newly diagnosed metastatic renal cell carcinoma had complaints of worsening vision and facial pain. Because there was a discrepancy in the pupillary size between his eyes, he underwent a computed tomography (CT) scan of the head, followed by craniofacial MR imaging, which revealed an enhancing mass in the right sphenoid sinus with adjacent extension. On neurological examination, he was noted to have complete hemifacial numbness and Horner's syndrome on the right side. MR imaging of the face demonstrated a homogeneously contrast-enhancing lesion centered within the right sphenoid sinus measuring 33 × 20 × 27 mm (Figure
Axial and coronal T1-weighted, gadolinium-enhanced MR imaging revealing a homogenously enhancing lobulated lesion centered within the right sphenoid sinus with extension into the carotid canal, pterygopalatine fossa, and optic nerve canal.
A 42-year-old woman with several weeks of frontal headaches initially presumed to be secondary to a sinus infection underwent MR imaging after antibiotic medications failed to alleviate her symptoms. Her neurological examination was nonrevealing except for mild gait instability. Brain MR imaging demonstrated a lobulated, 25 × 20-mm sellar and suprasellar lesion with extension into the right cavernous sinus and encasement of the right internal carotid artery. This lesion was isointense on T1- and T2-weighted images and heterogeneously enhancing with gadolinium administration. A CT scan was consistent with a lytic lesion involving the central skull base extending laterally and posteriorly to involve the bilateral medial sphenoid wings and the clivus, respectively. A transnasal approach was undertaken to obtain a biopsy of the lesion. Histopathologic evaluation was consistent with a diagnosis of diffuse large B-cell lymphoma (Figure
Axial and coronal T1-weighted MR imaging of the brain with gadolinium enhancement demonstrating a heterogeneously enhancing lobulated sellar and suprasellar lesion with extension into the right cavernous sinus and encasement of the right internal carotid artery.
A 53-year-old woman presented with a known diagnosis of breast cancer and a one-year history of worsening left eye vision with a more precipitous decline in the last month. MR imaging of the brain demonstrated a skull base lesion involving the left sphenoid bone, the anterior clinoidal process, and the cavernous sinus. On neurological assessment, the patient was noted to have an acutely diminished visual acuity in her left eye to a level of finger counting with left temporal visual field cut. MR imaging (not shown) showed a homogenously contrast-enhancing lesion of the skull base involving the greater sphenoid wing and the anterior clinoidal process with encasement of the optic nerve on the left. A left frontotemporal craniotomy was performed for biopsy of the lesion and to decompress the optic nerve and the cavernous sinus with a subtotal resection. Histopathological analysis confirmed the diagnosis of metastatic breast carcinoma. The patient did not experience improvement in her vision postoperatively. She underwent fractionated radiotherapy to the involved area. One year after surgery, her neurological findings were unchanged, and her systemic disease was under control.
Neoplasms originating from a multitude of sites have been reported to metastasize to the SPS region. Breast and lung cancer account for approximately two-thirds of SPS metastases, being the most common sources in women and men, respectively, [
The possible metastatic pathways to the pituitary and parasellar region include direct blood-borne metastasis to the posterior pituitary lobe, pituitary stalk, clivus, dorsum sellae, or cavernous sinus or leptomeningeal spread with involvement of the pituitary capsule [
Clinical symptomatology varies depending on the location of metastatic involvement. Cranial nerve palsies are the most frequent symptoms in cases of cavernous sinus metastases. These may be isolated, such as diplopia or ptosis, with the third (oculomotor) and the sixth (abducens) nerves being the most commonly involved, followed by the fourth (trochlear) nerve [
Because of their invasiveness, pituitary metastatic lesions have a high potential to cause visual deficits from suprasellar extension, with an incidence as high as 50% reported by Branch Jr. and Laws Jr. [
Symptoms strongly suggesting metastasis in the parasellar or sellar space include painful ophthalmoplegia in association with the sudden onset of DI [
Because pituitary adenomas also present with invasion of the sellar floor, cavernous sinus, or clivus, no specific neuroimaging criteria to define metastatic lesions in SPS region have been reported. The diagnostic imaging tools for SPS metastasis mainly include high-resolution CT and MR imaging. Although CT is superior to MR imaging in detecting the bone involvement, the latter is preferable to determine the relationship of the lesion to the surrounding neurovascular structures [
The management of SPS metastases is multimodal, including surgical resection, radiation therapy, and chemotherapy [
Besides its role as an adjunct after surgery [
Chemotherapy is commonly used alone or along with radiation therapy mostly for palliation in the treatment of metastatic disease in SPS region [
The prognosis of patients with metastases to the SPS region is grim in a majority of cases because of the aggressive character of the primary disease [
Sellar and parasellar metastatic lesions are relatively rare. Breast and lung have been reported to be the most common sources in both sexes. Suggestive symptoms include rapid onset of progressive ophthalmoplegia with retro-orbital or facial pain, visual impairment, and/or DI. Management varies depending on whether a primary source is identified, the symptomatology, the location and extent of the lesion, the stage of the primary disease, and the medical comorbidities. Subtotal or partial surgical resection is aimed mainly for symptom relief. A multimodal approach involving subtotal resection of the lesion followed by radiation and/or chemotherapy is widely accepted, especially in symptomatic patients whose primary disease is under control. Radiation with or without chemotherapy is generally recommended as first-line treatment in patients with advanced primary disease or in those with high-risk medical comorbidities. A biopsy usually precedes radiation therapy if the primary source of the metastasis is unknown. The prognosis for patients is generally poor, independent of the therapeutic modality, and the overall survival is less than two years.