|Year : 2018 | Volume
| Issue : 2 | Page : 168-171
A rare case of invasive pituitary tumor mimicking nasal mass
Inderdeep Singh1, Shruti Anil Sharma1, SGS Dutta2, Gurpreet Kaur3, D Phogat3
1 Department of ENT, Head and Neck Surgery, Institute of Naval Medicine, INHS Asvini, Mumbai, Maharashtra, India
2 Department of Neurosurgery, Institute of Naval Medicine, INHS Asvini, Mumbai, Maharashtra, India
3 Department of Pathology, Institute of Naval Medicine, INHS Asvini, Mumbai, Maharashtra, India
|Date of Submission||26-Aug-2018|
|Date of Acceptance||09-Nov-2018|
|Date of Web Publication||10-Jan-2019|
Shruti Anil Sharma
Department of ENT, Head and Neck Surgery, Institute of Naval Medicine, INHS Asvini, Colaba, Mumbai - 400 005, Maharashtra
Source of Support: None, Conflict of Interest: None
Pituitary apoplexy is a rare condition more often seen in large pituitary adenomas. It is preceded by an abrupt expansion of infarcted/hemorrhagic pituitary adenoma extending into the cavernous sinus or superiorly compressing the optic chiasm. An invasive pituitary tumor extends into the suprasellar cistern by stretching and fenestrating the diaphragma sellae and arachnoids layer. It is labeled as invasion of the cavernous sinus when the percentage of encasement of the internal carotid artery by the tumor is 67% or greater or for Grade 3 or 4 of Knosp classification. A 51-year-old male presented with headache, vomiting, and bleeding from the nose for 3-day duration and blurring of vision for 2-day duration. Contrast-enhanced magnetic resonance imaging of the brain revealed an enhancing sellar and suprasellar lesion with characteristic expansion into the cavernous sinus and extension into the sphenoid sinus and nasopharynx. The patient underwent endoscopic transnasal transsphenoidal excision of the mass followed by three-dimensional conformational radiotherapy and hormone replacement therapy with a favorable clinical outcome.
Keywords: Nasal approach, neuroendoscopy, sellar, suprasellar tumors
|How to cite this article:|
Singh I, Sharma SA, Dutta S, Kaur G, Phogat D. A rare case of invasive pituitary tumor mimicking nasal mass. J Mar Med Soc 2018;20:168-71
| Introduction|| |
Brougham et al. in 1950 first introduced the term pituitary apoplexy. Pituitary apoplexy is reported in 1%–25% of all pituitary adenomas. It is a rare but potentially lethal condition, more often seen in large pituitary adenomas.,, It is preceded by an abrupt expansion of infarcted or hemorrhagic pituitary adenoma extending into the cavernous sinus or compressing the optic chiasm superiorly., It characteristically presents as acute headache, visual deterioration, dysfunction of oculomotor nerves because of cavernous sinus compression, hypopituitarism, with or without altered consciousness. It may present infrequently with hydrocephalus and subarachnoid hemorrhage. It may present with subacute symptoms spanning hours to days before the full diorama of acute pituitary apoplexy is evident.,, An invasive pituitary tumor extends into the suprasellar cistern by stretching and fenestrating the diaphragma sellae and arachnoids layer. It is labeled as invasion of the cavernous sinus when the percentage of encasement of the internal carotid artery (ICA) by the tumor is 67% or greater or for Grade 3 or 4 of Knosp classification. Indications of conservative management are patients with minimal symptoms related to mass effect or those with major comorbidities. The approach to pituitary tumors is through a standard craniotomy or endoscopic or microscopic transsphenoidal route.
| Case Report|| |
A 51-year-old diabetic male presented to our center with headache, vomiting, bleeding from nose for 3-day duration and blurring of vision for 2-day duration. Diagnostic nasal endoscopy revealed a hemorrhagic mass in the right nasal cavity filling the posterior choana and extending into the nasopharynx [Figure 1]. Visual acuity of the right eye (RE) was finger counting at 2 m and the left eye (LE) was 6/18. Perimetry revealed bitemporal hemianopia. On fundoscopy, bilateral pale disc was seen.
|Figure 1: Preoperative nasal endoscopic image showing mass in the right nasal cavity filling posterior choana with areas of hemorrhage|
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Hormonal assays revealed insulin-like growth factor-I to be 276 ng/ml (normal: 87–238 ng/ml); serum prolactin levels >1000 ng/ml (normal: 2.10–17.70 ng/ml); serum luteinizing hormone <0.07 mIU/ml (normal: 1.5–9.30 mIU/ml); serum testosterone 10.13 ng/dl (normal: 241–827 ng/dl); and serum cortisol 0.89 mcg/dl (normal: 4.30–22.40 mcg/dl); whereas thyroid profile revealed T3, total to be 0.76 ng/ml (normal: 0.60–1.81 ng/ml); T4, total 6.30 mcg/dl (normal: 5.01–12.45 mcg/dl); and thyroid-stimulating hormone 1.28 uIU/ml (normal: 0.35–5.50 uIU/ml).
Contrast-enhanced magnetic resonance imaging (MRI) of the brain revealed an enhancing sellar and suprasellar lesion with characteristic expansion into the right cavernous sinus (Knosp classification Grade 4) with extension into the sphenoid sinus and nasopharynx [Figure 2] and [Figure 3].
|Figure 2: Sagittal view contrast-enhanced magnetic resonance imaging brain (preoperative) showing an enhancing sellar and suprasellar mass|
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|Figure 3: Coronal view of contrast-enhanced magnetic resonance imaging brain (preoperative) showing an enhancing sellar and suprasellar mass expanding into sphenoid and nasopharynx|
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The patient underwent endoscopic transnasal transsphenoidal excision of the mass. Hadad-Bassagasteguy flap was raised on the left nasal septum. Posterior part of vomer was removed to expose anterior face of sphenoid. Suprasellar tumor extending into sella, sphenoid, right nasal cavity, and nasopharynx was excised after sphenoidectomy. The tumor extending into the right nasal cavity, nasopharynx, and sphenoid was removed endoscopically by a rhinologist, and the sellar and suprasellar tumor was removed under endoscopic and microscopic guidance and sent for histopathological examination. Dural defect was covered with fat, Surgicel, and Gelfoam sandwich and secured with fibrin glue. Hadad flap was reposited over sphenoid and covered with fat and Surgicel.
Postoperatively, the patient's visual acuity improved to 6/18 in the RE and 6/9 in the LE. Nasal endoscopy on the 7th postoperative day revealed no evidence of cerebrospinal fluid leak, and the nasal cavity was healing well [Figure 4].
|Figure 4: Postoperative nasal endoscopy depicting well-healing nasal cavity|
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Histopathology revealed sheets of monotonous round cells separated by large areas of hemorrhage. Areas of fibrosis were interspersed with tumor trabeculae. Areas of necrosis were observed within the pituitary tissue. Reticulin stain revealed incompletely disrupted reticulin fibers [Figure 5] and [Figure 6]. Immunohistochemistry for proliferation markers for aggressive pituitary tumor was positive, with Ki-67 being positive and P53 being focally positive.
|Figure 5: High-power view showing sheets of monotonous round cells separated by large areas of hemorrhage|
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|Figure 6: Low-power view with reticulin stain: Incompletely disrupted reticulin fibers|
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Due to nonavailability of MRI, the patient underwent postoperative contrast-enhanced computed tomography head, which showed hypodense area with surrounding hyperdensity in sellar region [[Figure 7] and continuity of sellar floor with sphenoid sinus which was likely the postoperative skull base defect [Figure 8].
|Figure 7: Sagittal contrast-enhanced computed tomography head (postoperative) showing hypodense area with surrounding hyperdensity in sellar region|
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|Figure 8: Axial contrast-enhanced computed tomography head (postoperative) showing continuity of sellar floor with sphenoid sinus likely postoperative skull base defect|
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Postoperatively, the patient underwent adjuvant radiotherapy(#35,70 cGy) and hormone replacement therapy.
The patient is currently under regular follow-up by the neurosurgeon, rhinologist, and endocrinologist and remains symptom-free.
| Discussion|| |
Invasive macroadenoma with extension into the nasal cavity and nasopharynx is rare, and only a few cases have been reported in the literature. The reasons why some pituitary adenomas are more locally aggressive are unknown so far. Invasion into the sphenoid and the nasal cavity may manifest with varied symptoms. The patients with suprasellar tumors extending into nasopharynx may present as nasal obstruction, with/without epistaxis, and purulent rhinorrhea with/without visual loss. In such a scenario, other intracranial tumors, such as craniopharyngioma, chordoma, chondrosarcoma, meningioma, and sinonasal tumors need to be excluded. Hemoptysis, as was found in this case, could lead the investigations astray toward lung/gastrointestinal pathology. A high index of suspicion is therefore required for early diagnosis and facilitation of clinical intervention. The role of diagnostic nasal endoscopy in such cases is of paramount importance.
MRI is the gold standard for diagnosis and follow-up of pituitary adenomas. It clearly delineates the anatomical relationship of the tumor with the surrounding structures. The enhancement of the cavernous venous plexus by gadolinium diethylenetriaminepentaacetic acid provides the precise distinction between the medial, superior, inferior, and lateral compartments.
Classification of invasive pituitary adenomas by Knosp et al. is the widely accepted and used. They graded invasive pituitary adenomas on the basis of the relation of carotid lines with the limits of invasion. Medial, median, and lateral lines are drawn arbitrarily between supra- and intra-cavernous parts of ICA in the coronal view. Grade 0 denotes extension of tumor medial to the medial carotid line; Grade 1 – extension of tumor beyond the medial line but not up to the median line; Grade 2 – extension of tumor beyond the median line but tangent to the lateral line; Grade 3 – extension of tumor beyond the lateral line; and Grade 4 – tumor encases the intracavernous carotid artery.
Endoscopic endonasal transsphenoidal surgery (EETS) is being preferred as the first-line treatment modality for pituitary lesions. EETS is indicated in sellar, suprasellar, and selected invasive tumors. Strychowsky et al. and Schaberg et al. found that the extent of tumor resection was the same in the endoscopic and microsurgery technique., Schaberg et al. reported that the lesser operating time, brief hospital stay, and lesser blood loss in the endoscopic technique as compared to the microscopic technique. Strychowsky et al. in their study found that the visual outcomes of microscopic and endoscopic techniques were the same. Minet et al. concluded that nasal functions were better in endoscopic surgery. In spite of the favorable surgical outcome, in this case, it would be imprudent to conclude the efficacy of endoscopic over microscopic surgery from this study since both the methods were employed concurrently.
In our unique case, the patient initially presented with episodes of spurious hemoptysis which is extremely rare and not mentioned in literature so far. This could be attributed to the tumor extension into the nasopharynx which is a natural passage from sphenoethmoidal recess through the nasal cavity. The sudden deterioration of vision in case of suprasellar tumor prompted urgent MRI which confirmed the suspicion of the tumor compressing on the optic chiasm. The emergency transsphenoidal corridor approach taken for debulking the tumor led to restoration of vision. This highlights the role of early intervention in saving vision in such cases.
It has been our endeavor to emphasize the importance of sound clinical evaluation and multidisciplinary cooperation (neurosurgery, otorhinolaryngology, ophthalmology, and endocrinology) in the diagnosis and (neurosurgery, otorhinolaryngology, anesthesiology, endocrinology, and radiation oncology) in the management of a rare clinical entity through this case report.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
We would like to acknowledge the Departments of Neurosurgery, Ophthalmology, and Anesthesia for their technical support during surgery and Departments of Endocrinology for Hormone Replacement Therapy and Pathology for their support in processing the sample.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]