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Gastrointest Interv 2014; 3:116–119

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Gastrointestinal Intervention

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Case Report

Medulloblastoma metastatic to the pancreas: a case report

Hemanth Gavini,1 Venkat D. Arukala,1 John Stewart,2 Jeffrey H. Lee1,*

a b s t r a c t

We present a case of medulloblastoma metastatic to the pancreas in a 41-year-old woman with recurrent cerebellar medulloblastoma. On presentation,computed tomography of the abdomen and pelvis showed a 2.2 cm � 2.6 cm hypodense pancreatic head mass associated with obstruction of thepancreatic duct. Endosonography with fine needle aspiration (EUS-FNA) was performed. Cytology showed a malignant small cell neoplasm that wasconsistent with medulloblastoma and was morphologically identical to the primary cerebellar medulloblastoma. Metastatic medulloblastoma to thepancreas is an extremely rare form of a secondary pancreatic tumor and very few case reports exist in the literature. The diagnosis rests on comparingcytological features when primary and secondary tumors are both available.

Copyright � 2014, Society of Gastrointestinal Intervention. Published by Elsevier. All rights reserved.

Keywords: endosonography, fine needle aspiration, medulloblastoma, metastatic, pancreas

Fig. 1. The computed tomography scan shows a mass in the pancreatic head withpancreatic duct dilation (arrow).

Introduction

Medulloblastoma may account for up to 20% of central nervoussystem (CNS) tumors in the pediatric population, which makes itthe most common CNS malignancy in children.1 However, thisentity comprises only 0.4–1% of CNS neoplasms in adults.2 It isusually restricted to the CNS, and metastases often occur along theneuroaxis.3 Systemic (i.e., extraneural) metastasis is rare but por-tends a poor prognosis. Metastasis to the pancreas is relativelyuncommon, and usually diagnosed at autopsy in association withextensive metastasis to other organ systems.4,5 We report a case ofmetastatic medulloblastoma involving the pancreas that wasdiagnosed by endosonography with fine needle aspiration (EUS-FNA) in a woman with recurrent medulloblastoma.

Case Report

In 2009, a 41-year old African-American woman was admittedto our hospital with worsening severe occipital headaches and neckand back pain associated with difficulty in maintaining balance.Magnetic resonance imaging (MRI) of the head, showed a contrastenhancing mass in the posterior fossa. The patient underwentbilateral suboccipital craniotomy with total resection of the tumor,but had no ventriculoperitoneal shunt placed. The pathology of thetumor was consistent with medulloblastoma. She postoperativelydeveloped syndrome of inappropriate antidiuretic hormonesecretion (SIADH). She continued to have difficulty in maintainingbalance for which she had physical therapy. She subsequently

1Department of Gastroenterology Hepatology and Nutrition, University of Texas, M. D. Ande2Department of Pathology, University of Texas M. D. Anderson Cancer Center, Houston, TX,Received 4 September 2014; Accepted 21 September 2014* Corresponding author. Advanced Endoscopy Fellowship and Training, Department

Cancer Center, 1400 Pressler Street, Unit 1466, Houston, TX 77030-4009, USA.E-mail address: jefflee@mdanderson.org (J.H. Lee).

2213-1795/$ – see front matter Copyright � 2014, Society of Gastrointestinal Interventihttp://dx.doi.org/10.1016/j.gii.2014.09.007

underwent craniospinal radiation therapy with a plan to receivecisplatin and etoposide for three cycles, followed by eight cycles ofcyclophosphamide and vincristine. However, 1 week into her ra-diation therapy, she developed severe intractable nausea, vomiting,mucositis associated with odynophagia, and dysphagia. Shetherefore lost 30 pounds. Because of her poor performance status,

rson Cancer Center, Houston, TX, USAUSA

of Gastroenterology, Hepatology, and Nutrition, University of Texas, MD Anderson

on. Published by Elsevier. All rights reserved.

Fig. 2. The endosonography scan shows a heterogeneous mass in the head of thepancreas.

Fig. 3. Endosonography with fine needle aspiration of the pancreatic mass in pancreas.

Hemanth Gavini et al. / Medulloblastoma metastatic to the pancreas 117

adjuvant chemotherapy could not be initiated. Cerebrospinal fluid(CSF) analysis obtained by lumbar puncture did not reveal anymalignant cells.

One month later, a repeat MRI scan to evaluate worseningdysarthria and ataxia showed progression of the disease. She was

Fig. 4. Fine needle aspiration cytology (left) with Diff-Quik stain at �500 magnification ancohesive malignant small cell neoplasmwith neuroendocrine features (i.e., fine chromatin anDiff-Quik stain. Mitotic figures and apoptotic bodies are present.

subsequently started on chemotherapy with cisplatin, etoposide,cyclophosphamide and carboplatin. A repeat MRI revealed pro-gression of the disease with a significant increase in the size of thetumor. She then underwent a redo midline posterior fossa crani-otomy for excision of the tumor; its pathology was consistent withrecurrent medulloblastoma. This was complicated by a CSF leakfrom her incision, which required a repeat posterior fossa crani-otomy to indirectly repair the CSF leak and to place a lumbar spinaldrain. This was followed by intensity modulated radiation therapyto 40 Gy in 20 fractions.

Approximately 8 months later, she presented to the emergencycenter complaining of midepigastric pain with nausea and vomit-ing. Computerized tomography (CT) of the abdomen and pelvisshowed a 2.2 cm � 2.6 cm hypodense mass in the head of thepancreas and dilation of the pancreatic duct (Fig. 1). She underwentEUS-FNA on the 2.3-cm heterogeneous mass, which containedmultiple small anechoic spaces mixed with hyperechoic andhypoechoic areas in the head of the pancreas. The mass abutted theportal vein. Multiple hyperechoic strands with a lobular appear-ance were in the body and tail of pancreas (Figs. 2, 3). Cytologicalsmears and cell block sections with immunoperoxidase stainsshowed a malignant small cell neoplasm that represented an un-usual high grade primary pancreatic neuroendocrine carcinoma ora metastatic medulloblastoma (Figs. 4, 5). The absence of keratinexpression and the close morphologic similarity between thepancreatic aspirate and the histologic sections of the patient’srecurrent medulloblastoma favored metastatic medulloblastomaover a second primary high grade pancreatic neuroendocrineneoplasm. A positron emission tomography/CT scan showed mul-tiple osseous metastases and metastatic disease to the proximalpancreas. Informed consent was obtained from the patient for thisreport.

Discussion

This is the first case of medulloblastoma with pancreaticmetastasis diagnosed antemortem by EUS-FNA in the Englishliterature. After glioblastoma and meningioma, medulloblastoma isthe third most common CNS tumor to exhibit systemic metastases.In 1955, Weiss6 proposed rigid criteria for diagnosing extracranial

d (right) with Papanicolaou stain at �600 magnification. The smears show a looselyd inconspicuous nucleoli). The cells have scant cytoplasm that is best visualized on the

Fig. 5. Fine needle aspiration with cell block (�400 magnification). H&E staining shows malignant small cell neoplasm with necrosis. Immunoperoxidase stains are positive for theneuroendocrine markers chromogranin (diffuse), synaptophysin (focal), and CD56 (focal); and is negative for the keratins AE1/AE3 and CAM5.2. H&E, hematoxylin and eosin.

Table 1 Summary of Clinical Data of Previously Reported Patients with Me-dulloblastoma Metastatic to the Pancreas

Study Sex/age Tumortype

Recurrence/residual

disease in CNS

Extraneuralmetastasis

Paterson 195314 M/2 DM þ Bone marrowLymph nodesPelvisParotidsLiverPancreas

Rubinstein andNorthfield 196415

M/13 CM _ Bone marrowBonesThymusLymph nodesTestisPancreas

Lewis et al 197313 M/43 DM þ Bone marrowBonesLymph nodesLiver lung PancreasUreters

Kleinman et al 19815 F/26 DM þ Bone marrowBonesLymph nodesPleuraRetrosternal musclesSphenoid sinusAdrenal cortexLiverPancreas

Krouwer et al 19914 F/46 DM _ Pancreas

CM, cerebellar medulloblastoma; DM, desmoplastic medulloblastoma; F, female; M,male.

Gastrointestinal Intervention 2014 3(2), 116–119118

metastasis from primary CNS malignancies. These criteria include asingle histologically characteristic CNS tumor, a clinical historyindicating a primary CNS lesion, a complete postmortem exami-nation to exclude peripheral primary tumor, and similar histolog-ical findings between the CNS and peripheral lesions.6 In thispatient, all of these criteria were fulfilled antemortem.

After the initial diagnosis of medulloblastoma, the average in-terval to the development of extraneural metastasis is 18 months.In some patients, 13 years have elapsed after the initial diagnosisbefore metastasis developed.7 Our patient, who had a high risk formetastasis, survived 3 years prior to being diagnosed. Osseousmetastases (77%) are the most frequent site for extracranial spreadin adults, followed by lymph nodes (33%) lungs (17%), muscle (13%),liver (10%), and the pleura.

The spread of a primary CNS neoplasm extracranially occursprimarily by seeding via CSF pathway8 (which gives rise to subduralsubarachnoid metastasis9,10) and within the central canal of thespinal cord5 via a hematogenous route or lymphatic route. Reportshave also described spread by shunt tubing as a consequence oftumor cells escaping into the systemic circulation owing to thebreak in the blood–brain barrier. This potentially occurs as a resultof tumor surgery with the insertion of a cerebrospinal shunt.11,12

Metastasis to the bone and pancreas in our patient made hema-togeneous spread more likely.

To our knowledge there are only five reported cases4,5,13–15 inwhich medulloblastoma metastasized to the pancreas (Table 1). Infour of these cases, the diagnosis was made at autopsy withmetastasis to other systems. In one case report, the diagnosis wasmade antemortem by percutaneous needle biopsy.4 In our patient

Hemanth Gavini et al. / Medulloblastoma metastatic to the pancreas 119

with recurrent medulloblastoma, the diagnosis was made ante-mortem by EUS–FNA of the mass in the pancreas, which alloweddetailed evaluation of the pancreas and acquisition of tissue diag-nosis in one setting.

Conflicts of interest

All contributing authors declare no conflicts of interest.

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