Coexisting sellar Rathke cleft cyst and planum sphenoidale meningioma: illustrative case (original) (raw)

The sellar region, primarily occupied by the pituitary gland, is a common origin of heterogeneous types of primary tumors. Pituitary adenomas (PAs) constitute greater than 80% of tumors in this region and approximately 15% of all intracranial lesions.1, 2 Other neoplasms of the sellar region, in decreasing order of prevalence, include craniopharyngiomas (CPs), Rathke cleft cysts (RCCs), meningiomas, and chordomas.3

Meningiomas represent the most common primary intracranial tumors in adults, with incidences of 3.61% in males and 8.36% in females.4 The co-presentation of tumors in the sellar region and meningiomas in the sellar or parasellar areas is an exceptionally rare phenomenon.5 They could present either as collision tumors, defined as histologically distinct, intermixed tumors occurring in the same anatomical location, or as coexisting tumors, which can occur in the same or different anatomical locations.3

Although there have been reports of sellar lesions, particularly PAs, coexisting with meningiomas in distinct intracranial locations,[6](#bib6 bib7)–8 coexisting tumors comprising RCCs and adjacent meningiomas are exceedingly rare and have been reported only once.9

Herein, we report the case of a 49-year-old female patient who presented with headaches and was subsequently diagnosed with coexisting tumors composed of an RCC of the sellar region and a planum sphenoidale meningioma.

Illustrative Case

History and Examination

A 49-year-old female patient with a history of end-stage renal disease (ESRD) and kidney transplant 8 years earlier was referred to our medical center due to a severe headache. The headache had started 20 days prior and increased in intensity after her first hemodialysis session. Her medical history was otherwise unremarkable, with no history of radiation to the head and neck region.

Upon evaluation, she described the headache as a constant throbbing pain, primarily affecting the right side of the head with an intensity of 9/10 on the visual analog pain scale. The intensity did not change with different positions. The patient’s only other complaint was increased thirst. Her history and physical examination were otherwise normal. Notably, her preoperative visual examination was unremarkable, with no evidence of visual field defects. She did not experience nausea, vomiting, or decreased consciousness. The preoperative hormone panel revealed an insulin-like growth factor–1 (IGF-1) of 292 ng/mL (reference range 44–227 ng/mL) and a prolactin level of 70.7 ng/mL (reference range 2–25 ng/mL). Growth hormone, adrenocorticotropic hormone, and thyroid-stimulating hormone levels and urine specific gravity were within the reference range.

Magnetic resonance imaging (MRI) with contrast enhancement revealed two distinct lesions. One lesion was an 11 × 13 × 18–mm hypointense sellar mass, predominantly consistent with a pituitary macroadenoma with apoplexy due to the intrinsic T1 shortening, which raised suspicion for proteinaceous or hematogenous contents. The second lesion was a 19 × 27 × 22–mm homogeneously enhancing, extra-axial mass located in the right planum sphenoidale (Fig. 1A and B). This lesion exerted mass effect on the right optic chiasm and encased the right supraclinoid internal carotid artery (ICA) as well as the proximal segments of the left M1 and A1 arteries, resulting in luminal narrowing. These imaging characteristics are most consistent with a diagnosis of meningioma.

FIG. 1.

FIG. 1.

Preoperative axial (A), coronal (B), and sagittal (C) T1-weighted MRI with contrast enhancement illustrating the homogeneously enhancing planum sphenoidale meningioma (yellow arrows) and the heterogeneously enhancing sellar lesion (green arrowheads). Corresponding postoperative axial (D), coronal (E), and sagittal (F) MRI with contrast enhancement demonstrating complete tumor removal with sparing of the pituitary gland.

Operative Technique

Considering the debilitating headache and the patient’s treatment preferences, a two-stage resection was planned. During the first stage, the patient underwent an endoscopic transnasal transsphenoidal (TNTS) approach for resection of the intrasellar lesion. After appropriate access to, and visualization of, the pituitary sella, the dura was opened, and the lesion appeared to be a cystic lesion with a soft solid component intermixed with firmer normal gland tissue adjacent to the tumor. A cystic yellow-white component gushed out after opening the cyst, and a series of ring curettes were used to remove the solid component of the tumor along with the cyst wall in a piecemeal fashion.This process was repeated until maximal safe resection was achieved. Intraoperative frozen section revealed RCC remnants, which was later confirmed by histological examination (Fig. 2).

FIG. 2.

FIG. 2.

**Left:**Section from the patient’s sellar tumor resection shows amorphous eosinophilic material, which is consistent with RCC remnants. Right: Section from the patient’s right planum sphenoidale tumor resection shows atypical epithelioid cells forming whorls. The histological findings are consistent with a meningioma, WHO grade 1. Hematoxylin and eosin, original magnification ×10.

Twenty-eight days after the primary procedure, the patient subsequently underwent a right frontoparietal craniotomy for resection of the meningioma. Following the initial approach, the sphenoid bone was drilled down to the orbit to allow maximal access to the cavernous sinus region and the anterior cranial fossa. The dura was opened, and under the microscope two retractors were set up and used to elevate the frontal lobe, which allowed for exposure of the tumor. The orbitocranial skull base approach allowed us to come along the floor of the anterior cranial fossa to identify the skull base meningioma. The tumor appeared to originate from the anterior clinoid process on the right and extended along the dura of the cavernous sinus as well as the anterior cranial fossa. Image-guided navigation was used to confirm the location of the tumor. Tumor debulking was completed using a surgical aspirator. Dissectors were used to dissect the tumor away from the brain and optic nerves. The optic chiasm was identified and preserved, as were the carotid artery and third cranial nerve. The tumor appeared to be gray and soft and originated from the bone of the anterior skull base. The tumor was debulked in a piecemeal fashion until a gross-total resection, with the exception of the dural skull base, was achieved. Clinoidectomy was not performed due to the associated risk of injury to the ICA and optic nerves. Instead, we opted to cauterize the dura in the region of the clinoid and planum sphenoidale. Pathology of the second lesion revealed meningioma, World Health Organization (WHO) grade 1 (Fig. 2).

Outcome and Follow-Up

At the 2-month follow-up visit, the patient’s symptoms including headaches and increased thirst had resolved, and recovery was achieved without any neurological or vascular sequelae. Follow-up MRI at 6 months revealed gross-total resection of both tumors with sparing of the pituitary gland (Fig. 1C and D). IGF-1 levels remained elevated after surgery, which was attributed to the underlying kidney dysfunction.

The necessary informed consent was obtained in this study.

Discussion

Observations

The presentation of an RCC and meningioma as coexisting or collision tumors is exceptionally rare, with only one previously reported case involving a female patient who presented with vision changes and unilateral abducens nerve palsy. A sellar and suprasellar cystic lesion with bilateral invasion of the cavernous sinus was evident on MRI.9

In contrast, there have been several documented instances of PA and meningiomas occurring either as collision tumors or as separate entities not temporally or spatially related.5, 6, 8 A review of studies that have reported a sellar lesion such as PA, RCC, chordoma, or CP, along with a coexisting adjacent para- or suprasellar meningioma, is presented in Table 1.[9](#bib9 bib10 bib11 bib12 bib13 bib14 bib15 bib16 bib17 bib18 bib19 bib20 bib21 bib22 bib23 bib24 bib25 bib26 bib27 bib28 bib29 bib30 bib31 bib32 bib33 bib34 bib35 bib36 bib37 bib38 bib39)–40

TABLE 1.

Literature review of studies reporting primary sellar lesions coexisting with sellar and parasellar meningiomas

Authors & Year Sex Age (yrs) Pituitary Lesion Meningioma Location Op Approach No. of Ops Sxs FU Imaging & Outcome Notes
O’Connell, 196110 F 47 NF-PA Tuberculum sellae Pterional 1 Visual disturbances No FU imaging available; pt’s Sxs resolved
Kitamura et al., 196511 F 66 NF-PA Sphenoid wing Pterional 1 Visual defects, HA NA
Brennan et al., 197712 M 36 NF-PA Sphenoid wing Pterional 1 Blurry vision Rt eye vision deterioration; postop radiotherapy for pituitary lesion; no FU imaging available
Deen & Laws, 198113 M 65 CP Sellar region Pterional 1 HAs, intellectual deterioration NA
Yamada et al., 198614 F 52 NF-PA Sphenoid ridge Pterional 1 HA, visual disturbance, galactorrhea Baseline postop prolactin remained high; no FU imaging available
Zentner & Gilsbach,198915 M 46 PRL-PA Planum sphenoidale Pterional 1 Visual disturbance, oculomotor palsy No FU imaging; postop course uneventful
F 63 NF-PA Parasellar TNTS, pterional 2 Ataxia, bitemporal hemianopsia No FU imaging; postop course uneventful
F 61 NF-PA Sellar TNTS, pterional 3 HA, bitemporal hemianopsia 2nd & 3rd ops performed due to intertumoral bleed & pt deterioration
Görge et al., 199316 M 53 PRL-PA Para- & suprasellar Frontolat 1 Impotence, decreased libido, defective vision FU imaging NA; hormone levels remained elevated after op
Laun et al., 199317 F 61 NF-PA Tuberculum sellae NA NA Bitemporal hemianopsia, lt eye visual deterioration No recurrence at 2-yr FU; imaging data NA
Cannavò et al., 199318 F 47 GH-PA Retrosellar Pterional 1 Acromegaly, HA, amenorrhea Complete PA resection; partial meningioma resection achieved
Abs et al., 199319 F 47 PRL-PA Tuberculum sellae Subfrontal, TNTS 2 Aphasia, unilat hemiparesis, amenorrhea Sellar lesion could not be removed completely via pterional approach, mandating 2nd TNTS op
F 82 NF-PA Sphenoid ridge TNTS 1 Bitemporal hemianopsia Partial pituitary resection; FU imaging NA
F 51 NF-PA Sphenoid wing None 0 Palpebral edema, exophthalmos Meningioma treated w/ embolization
Canda et al., 200220 F 43 PRL-PA Sphenoid wing TNTS 1 Amenorrhea, galactorrhea Meningioma identified at time of op; FU imaging NA
Prevedello et al., 200721 F 52 NF-PA Tuberculum sellae TNTS 2 HA, rt temporal visual field loss 2nd op done due to presumed adenoma remnants
Lu et al., 200822 F 52 PA Tuberculum sellae TNTS, pterional 2 Blurry vision Craniotomy done to remove residual tumor
Della Puppa et al., 201123 F 81 CP Suprasellar Pterional 1 HA, bitemporal hemianopsia, visual loss Gross-total removal at time of op; no FU imaging; clinic FU revealed symptomatic improvement
Ramirez et al., 201224 F 61 NF-PA Ant clinoidal Pterional, TNTS 2 HA, cognitive decline, urinary incontinence, gait disturbance Meningioma incidentally found during op for neurocysticercosis cyst resection; minimal residual lesion after op
Mahvash et al., 201425 F 36 NF-PA Tuberculum sellae TNTS 1 Frontal HA & visual field defect of rt eye Complete lesion resection
Ruiz-Juretschke et al., 201526 F 61 NF-PA Planum sphenoidale TNTS 2 Progressive vision loss & bitemporal hemianopsia 2nd TNTS approach done to remove tumor remnants & repair CSF leak
Karsy et al., 201527 F 70 NF-PA Suprasellar TNTS 1 Altered mental status, mutism, incontinence Meningioma diagnosed after histological assessment; FU imaging NA
Lim et al., 201628 F 65 NF-PA Olfactory groove & tuberculum sellae TNTS 1 Visual disturbance, vertigo Persistent olfactory groove meningioma
Zhao et al., 201729 F 58 GH-PA Sellar region TNTS, craniotomy 2 Acromegaly, HA Craniotomy done due to tumor remnants
F 58 GH-PA Sellar region TNTS, craniotomy 2 Acromegaly, HA Craniotomy done due to tumor remnants
Amirjamshidi et al., 201730 F 37 PRL-PA Suprasellar Pterional 1 Oligomenorrhea, diplopia, HA, visual disturbance Pituitary lesion not surgically removed & treated w/ cabergoline
M 42 PA Suprasellar TNTS, pterional 2 Acromegaly, decreased visual acuity, bitemporal hemianopsia Vision failed to improve after TNTS tumor removal
Jamshidi et al., 20189 F 44 RCC Diaphragma sellae TNTS 2 Vision change, lt CN VI palsy Collision tumor diagnosed after op; 2nd op required due to RCC recurrence
Gezer et al., 202031 F 34 PA Sellar region EETS 1 Menstrual irregularities, proximal muscle weakness, weight gain Both tumors located in sellar region; total resection on FU imaging
Liu et al., 202032 M 43 CP Sphenoid crest NA 1 Blurry vision Pt lost to FU
M 64 CP Olfactory sulcus NA 1 Visual decline, fatigue, polyuria Pt lost to FU
Bao et al., 202133 F 62 PA Tuberculum sellae TNTS 1 Progressive visual loss in lt eye Complete resection of both tumors
F 56 PA CS Transmaxillary-transpterygoid 1 HA & bilat visual loss Meningioma discovered after pathological exam of resected tissue; partial tumor remnant in CS region
Hu et al., 202234 M 48 NF-PA Planum sphenoidale Pterional 1 HA FU imaging NA
F 52 NF-PA Sphenoid ridge Craniotomy 1 HA, progressive vision loss Growth of residual PA; radiotherapy done for recurred lesion
F 29 PRL-PA Petroclival Craniotomy 1 HA, amenorrhea Subtotal resection of petroclival tumor; FU imaging NA
De Vries et al., 202335 F 75 NF-PA Suprasellar w/ extension to rt CS EETS 1 Depression, fatigue, weight loss, bilat homonymous hemianopsia Remnant in CS; postop epistaxis requiring surgical management
Aydin et al., 202336 M 65 PA Olfactory groove Pterional, TNTS 2 HA & rt homonymous hemianopsia Small cystic lesion remnant in sella; minimal residual meningioma remnant at ant cerebral artery A1 segment
Geldres et al., 202337 F 52 NF-PA Lt CS Transzygomatic 1 Sz, throbbing HA, diplopia Complete excision achieved
Chatain et al., 202438 M 47 PA Tuberculum sellae Pterional 1 Progressive vision loss Residual enhancing lesion at floor of sella turcica
Baldawa & Raikhailkar, 202439 F 50 NF-PA Tuberculum sellae TNTS 1 Bifrontal HA, progressive vision decline Collision tumor located exclusively in sellar region
Salehipour et al., 202440 F 42 CP Olfactory groove & suprasellar Pterional 1 HA, visual impairment Small enhancing nodule remnant at pituitary stalk; meningioma completely excised
Present case F 49 RCC Planum sphenoidale TNTS, pterional 2 HA, excessive thirst Maximal resection achieved at 6-mo FU; sparing of pituitary gland

In our case, the presence of two distinct tumors was anticipated based on preoperative radiological imaging. While the sellar lesion was presumed to be a hemorrhagic PA based on epidemiological factors and hormonal evaluations, RCC also remained among our differential diagnoses. However, confirming the exact nature of the lesion preoperatively was deemed unnecessary, as it would not have altered the treatment approach, which was guided by the patient’s symptoms and preferences. Elevated IGF-1 and prolactin levels were later attributed to the underlying renal insufficiency.

The co-occurrence of distinct tumors in proximity or as collision tumors can be influenced by several factors, although the exact underlying etiologies remain obscure. Prior trauma, radiotherapy, or surgery could initiate a cascade of factors associated with de novo tumor growth.30 Additionaly, biochemical interactions associated with a specific tumor might trigger secondary tumor formation through the activation of growth signals and transcription in adjacent cells.30, 41 Genetic factors and the effect of pituitary hormones (e.g., growth hormone, prolactin) in the case of a functioning adenoma have also been proposed as contributors to the coexistence of two distinct tumors.42, 43 Alternatively, the presence of such tumors could be a mere coincidence, as the occurrence of meningiomas and adenomas is higher in the female population.40

The surgical approach to meningiomas varies by location, with some studies advocating for a single surgical procedure14, 21, 25 and others favoring two separate operations, using either the same approach26 or different approaches in separate sessions.36, 44 In some cases, due to similar lesion enhancements, it was not evident from preoperative imaging that a coexisting tumor existed. The observation of different consistencies during the operation or postoperative histological sampling examination revealed the presence of two distinct tumors, necessitating a second operation to remove the remnants of the secondary tumor.24, 33, 39

In our case, based on distinguishable enhancements, two separate lesions were anticipated. It was deemed appropriate to remove the sellar lesion in the first session and address the presumed meningioma in a subsequent operative session. This approach had two advantages: first, the TNTS approach for the pituitary lesion was less invasive than open craniotomy, allowing for faster recovery and earlier planning of the second surgery; second, addressing the presumed adenoma first would be more prudent in cases in which the effect of growth hormone or prolactin secretion from the adenoma could induce growth-stimulatory effects on the meningioma, as previous studies have suggested that the effect of growth hormone on arachnoid cap cells could contribute to meningioma development and growth.43 Through this approach, the stimulatory effects of growth hormone or prolactin would be potentially weaned off the coexisting meningioma, theoretically limiting further growth of the meningioma or its remnants until the second operative session.

Additionally, due to the size of the meningioma, encasement of major vessels as indicated by radiological findings, and the underlying hemostatic abnormalities commonly evident in patients with ESRD, we opted not to approach the lesion using the TNTS approach. This decision was based on the lower control over unexpected bleeding and the high probability of incomplete tumor removal associated with the TNTS approach. Also, this approach was inadequate for dissecting portions of the tumor located lateral to the ICA and optic nerve.

Complete resection of coexisting tumors using the TNTS approach is usually achieved in cases in which both lesions are confined to the sellar region.25, 31, 33 Lim et al.28 have noted that although an extended endoscopic transsphenoidal (EETS) approach might be feasible for anterior skull base meningiomas (in their case, the olfactory groove), the approach carries higher risks of complications, including anosmia, cerebrospinal fluid (CSF) leaks, and ICA or cavernous sinus bleeding.22 In another case reported by De Vries, a 75-year-old female patient was found to have a sellar lesion, which, due to suprasellar extension, was approached using an EETS approach, ultimately leading to the development of pneumocephalus and epistaxis requiring further intervention.35

Lessons

Prior radiation to the cranial region for the treatment of previous pituitary lesions is a well-established risk factor for the development of meningiomas in the region. In patients without prior radiation, hormonal secretions such as prolactin and growth hormone have been implicated in the development of coexisting meningiomas. Nevertheless, postoperative histological examination in our case revealed the lesion to be an RCC. The management of such coexisting tumors should take into account patients’ underlying comorbidities, demographic characteristics, tumor location and type, and preferences. A two-stage approach can help ensure maximal safe resection while limiting the need for future operations or interventions due to unprecedented tumor growth or recurrence due to remnants.

Disclosures

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Author Contributions

Conception and design: Chang, Akhavan-Sigari, Park, Persad. Acquisition of data: Akhavan-Sigari, Park, Chivakula, Theodros, Bharani, Emrich, Tayag. Analysis and interpretation of data: Chang, Tayag. Drafting the article: Akhavan-Sigari, Harary, Theodros. Critically revising the article: Chang, Akhavan-Sigari, Park, Harary, Theodros, Bharani, Hori, Persad, Lam. Reviewed submitted version of manuscript: Chang, Akhavan-Sigari, Park, Harary, Chivakula, Theodros, Bharani, Hori, Persad, Lam, Ustrzynski, Tayag. Approved the final version of the manuscript on behalf of all authors: Chang. Administrative/technical/material support: Chang, Akhavan-Sigari, Park, Chivakula, Theodros, Lam, Tayag. Study supervision: Chang, Park.

Correspondence

Steven D. Chang: Stanford University School of Medicine, Stanford, CA. sdchang@stanford.edu.

References