
==== Front
J Neurosurg Case Lessons
J Neurosurg Case Lessons
J Neurosurg Case Lessons
Journal of Neurosurgery: Case Lessons
2694-1902
American Association of Neurological Surgeons

39284231
10.3171/CASE23764
CASE23764
OncologyOncologyTumorTumorCase Lesson
A rare case of central nervous system pancreatoblastoma metastasis: illustrative case
Mastropasqua Vincenzo MD 1
Obersnel Marco MD 1
Stifano Vito MD 1
Gessi Marco MD, PhD 2
Cristiano Corsi Domenico MD 3
Doglietto Francesco MD, PhD 1
1 Departments of Neurosurgery, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy
2 Departments of Pathology, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy
3 Department of Oncology, Ospedale Isola Tiberina Gemelli Isola, Rome, Italy
Correspondence Vito Stifano: Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy. stifano89@gmail.com.
INCLUDE WHEN CITING Published September 16, 2024; DOI: 10.3171/CASE23764.

Disclosures Dr. Doglietto reported personal fees for consulting from Brainlab outside the submitted work.

16 9 2024
16 9 2024
8 12 CASE2376408 1 2024
11 3 2024
© 2024 the authors
2024
the authors
https://creativecommons.org/licenses/by-nc-nd/4.0/ CC BY-NC-ND 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/)

BACKGROUND

Pancreatoblastoma is a malignant neoplasm of the pancreas, occurring usually in children and rarely in adults. Treatment consists of surgery with a variable combination of adjuvant therapies. Liver metastases are common, whereas brain diffusion is exceptionally rare.

OBSERVATIONS

The authors report the case of a 42-year-old man with a 16-year history of metastatic pancreatoblastoma, previously treated with surgery, chemotherapy, and radiotherapy, demonstrating a partial response. He presented with headache and dizziness, and brain magnetic resonance imaging (MRI) showed a cerebellar lesion. A craniotomy was performed with complete tumor removal, and the postoperative course was uneventful. Brain MRI showed gross-total resection of the lesion, and the patient was discharged with an improvement of the preoperative symptoms. Histopathological analysis confirmed the diagnosis of metastasis from pancreatoblastoma. The patient received adjuvant stereotactic radiotherapy and showed further clinical improvement at the last follow-up.

LESSONS

Brain metastases from pancreatoblastoma are exceptionally rare and poorly described in the literature. There is no standard therapy for this condition; hence, patients usually undergo treatments similar to those for other central nervous system metastases. All the described patients have had good clinical outcomes yet short-term follow-ups; therefore, further investigations are needed to better understand the best treatments for this condition.

https://thejns.org/doi/10.3171/CASE23764

pancreatoblastoma
brain metastasis
CNS metastasis
central nervous system metastasis
ABBREVIATIONS

CNS = central nervous system
MRI = magnetic resonance imaging
pan-CK = pan-cytokeratin.
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pmcPancreatoblastoma is a malignant epithelial neoplasm of the pancreas, accounting for less than 1% of pancreatic neoplasms. It commonly occurs in childhood, whereas pancreatoblastomas in adults are exceptionally rare, with fewer than 100 cases reported in the literature. The mean age at diagnosis is 41 years, and there is a slight male predominance. The etiology is unknown.1

There are no established therapeutic guidelines for pancreatoblastoma. Treatment consists of resection with a variable combination of chemotherapy, radiotherapy, or targeted therapy.2 Malignant behavior is common in adult pancreatoblastoma. Approximately 59% of patients have metastases at the time of diagnosis or afterward. The liver is the most common site of metastasis, followed by the lymph nodes and lungs. Chest wall, breast, bone, and especially brain metastases are extremely rare. Here, we present the case of an adult patient with a history of pancreatoblastoma who developed a cerebellar metastasis.

Illustrative Case

A 42-year-old man had a known history of pancreatoblastoma, diagnosed 16 years earlier, and had been treated with corporal-caudal pancreatectomy, multiple cycles of chemotherapy, local radiotherapy, and chemoembolization for liver metastases, eventually demonstrating partial remission. He presented to the emergency department of another hospital, complaining of worsening headache and dizziness that were unresponsive to medical treatment. The neurological examination showed gait instability and horizontal nystagmus and normal motor function and cranial nerve function, apart from long-lasting hearing loss due to cisplatin toxicity.

The patient was later admitted to our hospital for further treatment. Brain magnetic resonance imaging (MRI) revealed a posterior fossa lesion (maximum tumor dimension 30 mm) involving the vermis and the left cerebellar hemisphere, surrounded by perilesional edema and causing obstruction of the fourth ventricle with subsequent incipient hydrocephalus (Fig. 1). The patient underwent a median suboccipital craniotomy for tumor removal. Intraoperatively, the tumor was yellowish and had a solid consistency as well as rich vascularization with a poor cleavage plane with the surrounding parenchyma. FIG. 1. Axial (A) and coronal (B) T1-weighted sequences with contrast showing a hyperintense, contrast-enhancing lesion that involves the vermis and extends to the left cerebellar hemisphere. Axial T2-weighted sequence (C) showing perilesional edema and fourth ventricle compression. Axial T2-weighted sequence (D) showing incipient hydrocephalus.

The postoperative course was uneventful. On postoperative day 1, the patient was moved from the neurosurgical intensive care unit to the ward, and he was able to stand up and walk. The postoperative neurological examination revealed improvement of the dizziness with persistent horizontal nystagmus, which gradually improved over time. Postoperative brain MRI showed gross-total resection of the lesion without any complications (Fig. 2), and the patient was discharged on postoperative day 6 on tapering dexamethasone. FIG. 2. Axial (A), sagittal (B), and coronal (C) T1-weighted sequences with contrast showing gross-total resection.

Histopathological analysis of the resected tissues showed an epithelial-appearing neoplasm with geographic, hypercellular, solid tumor lobules separated by fibrous bands (Fig. 3 left). Scattered squamoid nests were present (Fig. 3 left inset). The tumor cells were strongly positive for pan-cytokeratin (pan-CK; AE1/AE3, Dako-Agilent; Fig. 3 right) and showed neuroendocrine differentiation with scant and patchy positivity for synaptophysin and chromogranin. The tumor cells displayed nuclear positivity for beta-catenin (Roche-Ventana; Fig. 3 right inset). FIG. 3. Left: The tumor was composed of solid nests separated by fibrous bands (hematoxylin and eosin staining) with foci of squamous metaplasia (inset). Right: The tumor cells were strongly pan-CK+ (immunostaining with pan-CK AE1/AE3 antibody) and showed nuclear accumulation of beta-catenin (inset, immunostaining with beta-catenin antibody). Bar = 10 μm (left) and 4 μm (right and insets).

One month after surgery, brain MRI showed no residual or recurrent tumor. The patient underwent adjuvant stereotactic radiotherapy with a total dose of 2500 cGy in 5 fractions. At the last follow-up, the patient showed further improvement in gait.

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

Observations

Brain metastases from pancreatoblastoma are exceptionally rare and poorly described in the literature. To date, only 3 case reports have been published. Szerlip et al.3 were the first to describe brain pancreatoblastoma metastases. They described the case of a 7-year-old girl who had undergone pancreatic surgery for pancreatoblastoma, followed by chemotherapy. One year after surgery, she developed a generalized seizure. Brain MRI showed diffuse intracranial disease, including contrast-enhancing lesions in the right cerebral peduncle and inferior frontal lobes. She underwent a right frontal lesion resection, with pathological confirmation of pancreatoblastoma. She then started systemic chemotherapy treatment, with stability of the disease at the 2-month follow-up.

The other 2 reports described an adult patient with pancreatoblastoma brain metastases. Snyder et al.4 detailed the surgical and radiotherapeutic management, whereas Elghawy et al.5 focused on the previous medical treatment, including an autologous hematopoietic cell transplant. The patient was a 28-year-old woman with a history of pancreatoblastoma, which had been diagnosed 5 years earlier by means of a pancreatic biopsy and was treated with chemotherapy and an autologous hematopoietic cell transplant, obtaining a complete response. She presented with acute-on-chronic headaches refractory to common medical therapy, dizziness, and blurry vision. Brain MRI revealed an enhancing left cerebellar lesion, and the patient underwent excision. Pathological examination confirmed the diagnosis of pancreatoblastoma metastasis, and the treatment was completed with Gamma Knife radiosurgery. Three months after surgery, the patient showed neurological improvement, and brain MRI did not show any intracranial disease.

Even if there are important differences between our patient and the pediatric patient,3 there are several similarities with the adult patient.4, 5 In both cases, there was a single cerebellar metastasis discovered in patients who had already undergone aggressive systemic treatment and after long disease-free periods (2 years in our patient, 5 years in the other). Because of the rarity of brain metastases from pancreatoblastoma, there is no standard treatment; hence, in all reported cases, patients underwent treatments similar to those for other central nervous system (CNS) metastases. All the described surgeries were uneventful, and radiotherapy was deemed necessary, given the radiosensitivity of pancreatoblastoma1, 6 and the unknown behavior of this condition.

Limitations

To date, this is the third case report of CNS pancreatoblastoma metastasis and the second one in an adult. All the reported patients have had good clinical outcomes but also a short-term follow-up after surgery. The rarity of this condition and the short duration of the postoperative follow-up do not allow us to draw definitive conclusions about the optimal management of these patients. Further investigations and longer follow-ups are needed to better understand the evolution and best treatments for this pathology.

Lessons

We report the case of a patient with brain metastasis from pancreatoblastoma, outlining the clinical history and the relevant neuro-oncological features of this rare condition. Notwithstanding the rarity of CNS spreading, patients affected by pancreatoblastoma should be monitored over a long period in consideration of the possibility of late-occurring metastasis. Multimodal treatment including surgery, radiotherapy, and systemic therapy appears appropriate, as in other CNS metastases.

Disclosures

Dr. Doglietto reported personal fees for consulting from Brainlab outside the submitted work.

Author Contributions

Conception and design: Stifano, Obersnel, Doglietto. Acquisition of data: Mastropasqua, Obersnel, Gessi, Corsi, Doglietto. Analysis and interpretation of data: Mastropasqua, Obersnel. Drafting the article: Mastropasqua, Obersnel. Critically revising the article: Stifano, Obersnel, Doglietto. Reviewed submitted version of manuscript: Stifano, Obersnel, Corsi, Doglietto. Approved the final version of the manuscript on behalf of all authors: Stifano. Administrative/technical/material support: Stifano, Doglietto. Study supervision: Doglietto.

Correspondence

Vito Stifano: Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy. stifano89@gmail.com.
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References

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