This case demonstrates the rare coexistence of a prolactinoma with craniopharyngioma and documents its radiological growth. This case suggests that patients with pituitary neoplasms should be followed closely and although prolactinomas can often be managed medically, a coexistent other lesion may require surgery for histological assessment and to reduce mass effect

Losa, Marco

MARIOTTI, STEFANO

Mortini, Pietro

SABA, LUCA

PubMed

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CASE REPORTThe birth and rise of a craniopharyngioma: the radiologicalevolution of an incidental craniopharyngioma detected onserial MRI during medical treatment of amacroprolactinomaStefano Mariotti1, Marco Losa2, Pietro Mortini2 & Luca Saba31Endocrinology, Department of Medical Sciences, University of Cagliari, Cagliari, Italy2Department of Neurosurgery, San Raffaele Scientific Institute, Vita-Salute University, Milan, Italy3Radiology, Department of Medical Sciences, University of Cagliari, Cagliari, ItalyCorrespondenceLuca Saba, Radiology, Department of MedicalSciences, University of Cagliari, Cagliari,09100 Italy. Tel:/Fax: 0706753193;E-mail: lucasaba@tiscali.itFunding InformationNo sources of funding were declared for thisstudy.Received: 11 October 2015; Revised: 21March 2016; Accepted: 3 May 2016doi: 10.1002/ccr3.623Key Clinical MessageThis case demonstrates the rare coexistence of a prolactinoma with cranio-pharyngioma and documents its radiological growth. This case suggests thatpatients with pituitary neoplasms should be followed closely and although pro-lactinomas can often be managed medically, a coexistent other lesion mayrequire surgery for histological assessment and to reduce mass effect.KeywordsChiasmatic cistern, craniopharyngioma, magnetic resonance, prolactinomasellar cavity.We would like to present the case of a 38-year-old femalepatient with amenorrhea, galactorrhea, and markedhyperprolactinemia discovered few months ago, with agiant macroprolactinoma and the unusual finding of analmost synchronous rise of a craniopharyngioma, duringthe progressive shrinking of the pituitary tumor on caber-goline therapy. Thus, in this patient, two uncommonconditions occurred: firstly, the sequence of the “birth” ofa craniopharyngioma and secondly, the presence of twosellar lesions at the same time.Five Magnetic Resonance Imaging (MRI) exams wereperformed and all the procedures were acquired on aGyroscan 1.5-T superconducting magnet (Philips, Best,the Netherlands) with a head coil before and after theadministration of 6 mL of contrast material (gadolinium,Gadovist, Bayer-Schering, Leverkusen, Germany).The patient underwent the first MRI for amenorrhea(performed 6 months earlier, after interruption of com-bined estrogen-progestin pill), galactorrhea, and markedhyperprolactinemia (>318,000 mIU/L [>15,000 ng/mL],normal values in our Laboratory: <424 mIU/L [<20 ng/mL] in women and <318 mIU/L [<15 ng/mL] in men).No other abnormalities of anterior and posterior pituitaryhormone secretion were documented. The patientreported mild cephalalgia since several years (for whichno specific investigation was performed), a computerizedcampimetry was normal and no other visual defects werepresent. A big (61 9 53 mm) mass with homogenoushyper-signal after the intravenous administration of con-trast material was documented in the sellar area, includ-ing the caudal part of the infundibular stalk, withoutinvolvement of the optic chiasm (Fig. 1A). A completeencasement of the internal carotid artery was also presentwith cavernous sinus invasion. These MRI features,together with the clinical presentation and the prolactinlevels, were indicative for macroprolactinoma and accord-ing to current guidelines, [1] the patient started cabergo-line, 1.5 mg/week. In the second MRI exam performedafter 2 months, serum PRL was 1490 mIU/L (70.3 ng/mL), galactorrhea disappeared and a reduction in theª 2016 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use anddistribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.1volume of the prolactinoma was clearly visible (Fig. 1B);two months after, normal menses reappeared. From thethird exam (performed after 7 months), besides furtherprogressive reduction in the prolactinoma (serum PRL496.1 mUI/L [23.4 ng/mL]), MRI revealed, in theinfundibular stalk, a small round lesion with a homoge-nous hypointense signal on T1 sequences and enhancedperipherally after gadolinium; these are typical MRI fea-tures associated with a craniopharyngioma (Fig. 1C).During the two following MRI checks (14 months and22 months), the stalk mass becomes larger reaching adiameter of 15.6 mm (from a value of 3.2 mm) with cra-nial dislocation of the optic chiasm (Fig. 1D and E).Indeed, the last MRI was performed 4 months in advancefor the sudden appearance about 3 weeks before of anim-portant visual field defect, which was confirmed by com-puterized campimetry. Further reduction in theprolactinoma size was observed, while serum PRL was207.8 mIU/L (9.8 ng/mL). Moreover, the patient hadregular menses and no clinical/biochemical evidence ofanterior or posterior pituitary failure. The patient under-went surgical procedure (by transphenoidal approach)that confirmed the diagnosis of craniopharyngioma (his-tological examination: papillary craniopharyngioma,Grade I WHO). The surgery was followed by anteriorpanhypopituitarism and central diabetes insipidus, requir-ing adequate substitution therapy with hydrocortisone,levothyroxine, estrogen-progestin pill, growth hormone,and desmopressin. Since no specific effort was made toremove the prolactinoma (very small and barely detect-able at the time of transphenoidal surgery), the patientwas kept on cabergoline therapy, with serum prolactinremaining <212 mIU/L (<10 ng/mL). Visual fieldimproved, but the recovery was not complete. The MRIcheck performed 2 months after the surgical procedure(Fig. 1D and F) did not find any evidence of recurrencefor both craniopharyngioma and prolactinoma, butshowed a herniation of the chiasmatic cistern into the(A) (B) (C)(D) (E) (F)Figure 1. Magnetic Resonance (MRI) coronal image TSE - T1-weighted after administration of contrast material. In panel A, B, C, D, and E theimages acquired in the first exam, after 2 months, after 7 months, after 14 months, and after 22 months, respectively, are given. The asterisksidentify the carotid arteries in the supra-clinoid segment. In panel A, the caudal part of the infundibular stalk (white arrow) seems to be involvedby the prolactinoma, whereas the optic chiasm does not show pathological signal (white arrowhead). There is also a complete encasement of theinternal carotid artery. In the panel B, the infundibular stalk (white open arrow) does not show alteration, whereas in the panel C, a small (mm3.2) hypointense mass with peripheral enhancement is observed; these are the typical features of a craniopharyngioma that become clearer in thepanel D with a mass diameter of 5.7 mm and panel E with a mass diameter of 15.6 mm. In the panel E, the cranial dislocation of the opticchiasm is also visible (white open arrowhead). In the MRI check, acquired 2 months after the surgical procedure, there was no evidence ofrecurrence of pathology (panel F) but the herniation of the chiasmatic cistern into the sellar cavity is visible (white dashed line).2 ª 2016 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.Macroprolactinoma with incidental craniopharyngioma S. Mariotti et al.sellar cavity, which could be responsible for the incom-plete recovery of visual field.Prolactinomas make up the majority of pituitarymacroadenomas and are fortunately often responsive tomedical management [2], as documented in the presentcase. In cases where the tumor is refractory to medicalmanagement, surgical resection is necessary [3]; sincerefractoriness to medical therapy is more frequent in giantprolactinomas (as the tumor of the present report) whichmay require multimodal therapy [4], we performed fre-quent MRIs to strictly follow the tumor morphology;however, the response to cabergoline therapy in terms ofreduction in tumor mass, prolactin secretion, and clinicalsymptoms was excellent.Magnetic resonance imaging is considered the imagingtechnique of choice in pituitary and para-sellar tumorsbecause it accurately depicts the complex anatomy aroundthe sellar well [5]. Several pathologic entities occur in thisregion, and most can be differentiated with MRI. Theseentities can be broadly divided on the basis of theirinvolvement, as follows: intra-sellar, infundibular, supra-sellar, anterior part of the third ventricle and/or optic chi-asm, and sphenoid and/or cavernous sinus.In this case, the extremely uncommon finding was theopportunity to identify the birth and rise of a cranio-pharyngioma, during the follow-up of a macroprolacti-noma responding to cabergoline therapy. Moreover, asecond uncommon condition was documented: the pres-ence of two different sellar lesions (prolactinoma andcraniopharyngioma) at the same time. This extremelyuncommon condition was probably responsible for thedelayed recognition of the craniopharyngioma image,which was detectable several months before the onset ofvisual field loss (Fig. 1C and D) and whose detectioncould have allowed earlier surgical removal.Finally, we considered the possibility that the appear-ance of the craniopharyngioma could be the consequenceof cabergoline therapy, but we feel it very unlikely,because until now no association has been describedbetween this drug and development of craniopharyn-gioma.Craniopharyngiomas are a benign but aggressive neo-plasms arising along the craniopharyngeal duct. MRI fea-tures suggest this pathological entity even if false-positiveresults may occur with cysts, metastases, infarctions, epi-dermoid cysts, and abscesses. All these lesions may appearas areas of low-signal intensity after the administration ofgadolinium-based contrast agent, but the rim enhance-ment is more likely associated with the craniopharyn-gioma [6].Some authors found that patients with craniopharyn-gioma occasionally also have prolactinomas and in 1986Wheatley et al. described the case of a 61-year-old manusing the CT image [7]. In a recently published reviewpaper, Guaraldi et al. reported that the associationbetween craniopharyngioma (typically adamantinomatoustype) and pituitary adenoma is very rare [8] but thiscondition can occur and therefore, it is important tostrictly follow imaging techniques of patients with pitu-itary tumors in order to identify the occurrence of a sec-ond neoplasm. Aside from these sporadic reports, to thebest of our knowledge, no condition has been identifiedso far with a clear association between prolactinomasand craniopharyngiomas. Given the relatively young ageof the patient, the possibility of a still unrecognizedgenetic mutation in one of the transcription factorsinvolved in the Rathke pouch’s formation and in theearly anterior pituitary development can be envisaged,although at the present time, this possibility remainsfully speculative.However, in this case also the location of cranio-pharyngioma is uncommon, because this tumor is fre-quently identified in the supra-sellar region only when itsdiameter is much bigger than one in our case. The obser-vation of the pituitary stalk craniopharyngioma is unusualbecause it is uncommon that this tumor may be diag-nosed at such an early stage that it only involves the pitu-itary stalk, before enlarging and extending to supra- orpara-sellar region [9].From the MRI point of view, in the differential diagno-sis setting, the primary tumors that can involve the pitu-itary stalk include gliomas, metastases (usually frombreast and lung cancers), leukemia (chronic myelogenousleukemia and acute myelogenous leukemia), lymphoma,and benign tumors like meningioma. However, when apatient develops a new pituitary stalk lesion, the physicianshould be aware that not infrequently these are malignantlesions and it is therefore often required to intervene sur-gically to obtain definitive histology [10].The present case suggests that physicians should bealerted to incidental diagnoses and that there is the needfor follow-up imaging and although prolactinomas canoften be managed medically, a coexistent other lesionmay require surgery for histological assessment and toreduce mass effect.Conflict of InterestNone declared.References1. Melmed, S., F. F. Casanueva, A. R. Hoffman, D. L.Kleinberg, V. M. Montori, J. A. Schlechte, et al. 2011.Diagnosis and treatment of hyperprolactinemia: anEndocrine Society clinical practice guideline. J. Clin.Endocrinol. Metab. 96:273–288.ª 2016 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd. 3S. Mariotti et al. Macroprolactinoma with incidental craniopharyngioma2. Schlechte, J. A. 2003. Clinical practice. Prolactinoma. N. Engl.J. Med. 349:2035–2041. Review. PubMed PMID: 14627789.3. Bloomgarden, E., and M. E. Molitch. 2014. Surgicaltreatment of prolactinomas: cons. Endocrine 47:730–733.4. Moraes, A. B., C. M. Silva, L. Vieira Neto, and M. R.Gadelha. 2013. Giant prolactinomas: the therapeuticapproach. Clin. Endocrinol. (Oxf) 19:447–456.5. Hess, C. P., and W. P. Dillon. 2012. Imaging the pituitaryand parasellar region. Neurosurg. Clin. N. Am. 23:529–542.6. Molla, E., L. Martı-Bonmatı, A. Revert, E. Arana, F.Menor, R. Dosda, et al. 2002. Craniopharyngiomas:identification of different semiological patterns with MRI.Eur. Radiol. 12:1829–1836.7. Wheatley, T., J. D. Clark, and S. Stewart. 1986.Craniopharyngioma with hyperprolactinaemia due to aprolactinoma. J. Neurol. Neurosurg. Psychiatry 49:1305–1307.8. Guaraldi, F., N. Prencipe, V. di Giacomo, M. Scanarini, V.Gasco, M. P. Gardiman, et al. 2013. Association ofcraniopharyngioma and pituitary adenoma. Endocrine44:59–65.9. Figueiredo, E. G., L. C. Welling, J. W. de Faria, and M. J.Teixeira. 2011. Pituitary stalk craniopharyngioma. BMJCase Rep. 2011: pii: bcr0620103097.10. Ariel, D., H. Sung, N. Coghlan, R. Dodd, I. C. Gibbs, andL. Katznelson. 2013. Clinical characteristics and pituitarydysfunction in patients with metastatic cancer to the sella.Endocr. Pract. 19:914–919.4 ª 2016 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.Macroprolactinoma with incidental craniopharyngioma S. Mariotti et al.

The birth and rise of a craniopharyngioma: the radiological evolution of an incidental craniopharyngioma detected on serial MRI during medical treatment of a macroprolactinoma

The birth and rise of a craniopharyngioma: the radiological evolution of an incidental craniopharyngioma detected on serial MRI during medical treatment of a macroprolactinoma

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