A STRANGER IN THE BACKYARD

PANCREATOBLASTOMA

A rare Case Report and Review of Literature

Dr Raghavendra Babu J, Dr Subash K.G, Dr Farman Ali, Keerthana Ganesh

Health Care Global Hospitals, Bangalore, India.

Corresponding Author: Dr Farman Ali; Department of HPB Oncology and Liver Transplant Surgery,

Room 222, 2nd floor, Tower 4, HCG Hospital, K.R Road, Sampangiramnagar, Bangalore – 560027

fali8042@gmail.com, Ph. +91 9739670031

Acknowledgements:
 DEPARTMENT OF PATHOLOGY AND TRANSLATIONAL MEDICINE
 DEPARTMENT OF DIAGNOSTIC RADIOLOGY
 DEPARTMENT OF ANESTHESIA AND CRITICAL CARE
 DEPARTMENT OF PEDIATRICS

ABSTRACT
The term Pancreatoblastoma was introduced in 1977 by Horie et al. to describe a rare
pancreatic tumor, previously known as infantile carcinoma of the pancreas, which shows
histological features similar to the pancreatic tissue at approximately the 7th fetal week. (1)
Pancreatoblastoma has multidirectional differentiation and is of embryonic primordium. (2)

Diagnosis of this upper abdominal mass is rarely, if ever, made preoperatively, and other more
common conditions are generally considered in differential diagnosis like neuroblastoma,
hepatoblastoma, malignant lymphoma, pseudo cyst and Wilms’ tumor. (3)

We, hereby report a rare case of a 5 year old Master M, with the radiological and clinical
features of a Pancreatoblastoma, focusing on the management of such disease. Our patient
was managed with surgical resection and no chemo radiation. Histopathology post operatively
confirmed a Pancreatoblastoma and allowed us to follow a realistic management protocol in
this rare and aggressive tumor.

This tumor occurs in children less than 10 years of age and the median age in adults is 37
years (range, 18–78 years), and men and women are equally affected.

Apart from surgical resection, optimal treatment has not been established.
Chemotherapy and radiotherapy may have a role in recurrent, residual, unresectable and
metastatic disease but the published data are limited [4].

The outcome in children is more favorable than in adults. The longest survival time reported,
following resection in a child was 28 years and an adult with Pancreatoblastoma was 9 years
(5).

KEYWORDS: Pancreatoblastoma, infantile carcinoma, primordium, pancreatic resection.

INTRODUCTION
Pancreatoblastoma is a rare malignant tumor of the pancreas originally reported by Bohn in
1885. Pancreatoblastoma has multidirectional differentiation and is of embryonic primordium.
(2)

Pancreatic carcinomas are classified on basis of cell of origin into: (a) ductal cell (b) islet cell (c)
Acinar cell and (d) uncertain histogenesis.

Except for islet cell tumor, the three year survival of pancreatic tumor is 2%
Notable exception to this prognostic statistics is a tumor of uncertain histogenesis that was
variously reported as Pancreatoblastoma or Infantile type pancreatic carcinoma.

The terminology is unacceptable to some because histologically this tumor does not have a
typical blastemal appearance and it has good prognosis.
Pancreatoblastoma is a rare tumor comprising 0.5% of epithelial neoplasm of pancreas.

It is actually the most common pediatric pancreatic neoplasm, more frequent in males than
females with a ratio of 2.1.

Though no age prevalence has been reported in previous reports recent studies show that
mean age at presentation is about 6 years.

Some rare cases have been described during early infancy and in adults.
The tumor is more common in the Asian (two third of cases) than in the white population.

Diagnosis of this upper abdominal mass is rarely, if ever, made preoperatively, and other more
common conditions are generally considered in differential diagnosis like neuroblastoma,
hepatoblastoma, malignant lymphoma, pseudo cyst and Wilms’ tumor. (3)

This tumor occurs in children less than 10 years of age and the median age in adults is 37
years (range, 18–78 years), and men and women are equally affected.
Apart from surgical resection, optimal treatment has not been established.
Chemotherapy and radiotherapy may have a role in recurrent, residual, unresectable and
metastatic disease but the published data are limited [4].

The outcome in children is more favorable than in adults. The longest survival time reported,
following resection in a child was 28 years and an adult with Pancreatoblastoma was 9 years
(5).

CASE REPORT
Here is a 5 year old patient, Master M, hailing from Yemen who presented with chief
complaints of pain abdomen since 3 months. The pain was of a dull nature and is
diffuse vague abdominal pain that gradually localised more towards the upper
abdomen. He also noticed a swelling over the upper abdomen which gradually
increased in size over the last three months.
 No h/o yellowish discolouration of skin, eyes, urine.

No h/o loss of appetite, loss of weight

No h/o fever, chest pain

No h/o growth and developmental delay

Vaccinations up to date.

No co-morbidities.

No significant family history.

On Examination;

A moderately built and nourished child, conscious and co-operative in nature.

No Pallor; No Icterus; No Lymphadenopathy; No clubbing; No pedal edema

Pulse Rate– 94/ min

B.P – 100/60 mm hg

CVS – S1, S2 heard no murmurs.

RS – B/L Normal Vesicular Breath sounds +

CNS – Developmental milestones attained normally.

No abnormality detected.

Per Abdomen: Mass ++ involving upper abdomen, cm;

Soft, non-tender; Bowel sounds +

FIGURE 1:
 Investigations:

 PET - CT Scan –

 9x12x11cm metabolically active lesion in neck and body of the pancreas with well-
preserved fat planes.
 No significant regional lymphadenopathy or distant metastasis.

 Routine blood investigations, Tumor markers, LDH, LFT, HIV and HEP Serology.

Significant findings:

 Hb – 11.5
 LDH – 232
 T.Bil – 0.2
 ALP – 258
 AFP – 273
 Beta HCG - <2.39
PLAN OF ACTION
A provisional diagnosis of Pancreatoblastoma was made based on the
radiological and clinical features. The tumor seemed surgically resectable and
therefore a decision in favor of surgical management was taken, after a multi-
disciplinary tumor board meeting. Hence, the patient was posted for a ‘Spleen-
preserving Subtotal Pancreatectomy’ in view of his young age and the high risk
of OPSI in splenectomised children.

FIGURE 2:

PRE-OPERATIVE IMAGE OF PANCREATOBLASTOMA

SURGERY
Under general anesthesia, we began with a transverse incision for laparotomy.

STEP 1:

Look for metastatic lesions.

There was no evidence of liver or distant metastasis.

STEP 2:

Obtaining access to the pancreas.

Lesser sac was opened through the gastro colic omentum followed by division of the
Gastrohepatic omentum.

The lesion in the pancreas was in the proximal body of the pancreas. It was about 15cmx
12cms in size.
STEP 3:

Mobilization of pancreas and adjacent structures.

The inferior border of body of the pancreas was mobilized.
Lymph nodes along the CHA and mesentery were removed.
Splenic vein and splenic artery were identified and protected.

STEP 4:

Resection of the lesion with macroscopically negative margins.

A tunnel was created behind the neck of the pancreas, anterior to splenic vein.
Pancreas was stapled at the neck using a vascular stapler (Signia).
Mobilization was carried out and the large veins draining the tumor which were joining portal
vein and SMV were ligated and divided.

STEP 5:

Extraction of specimen and closure.

Specimen was extracted out along with body and tail of pancreas.
Hemostasis secured. Closure done in layers.

SIGNIFICANT FINDINGS:
• A 15X12 cm mass in the body of pancreas, in its proximal part. 

• No evidence of distant metastasis. 

• Lymph nodes along the Common hepatic artery and mesentery were removed. 

POST-OPERATIVE COURSE:

o The patient was extubated on table and shifted to the ICU post-
operatively.
o He showed significant clinical improvement on POD 1, therefore, was
shifted to the ward and soft diet started.
o Ambulation was initiated on POD 2 and the abdominal drain was
removed on POD 3 as the output was almost none.
 o He was discharged in a hemodyanamically stable condition on POD 4 and
is on regular follow up.
o Adjuvant Chemotherapy was not considered as it was an R0 resection
and the patient’s parents returned to Yemen to come back after 3
months for a repeat PET – CT scan and follow up.

FIGURE 3:

Sheets and Acinar structures lined by monotonous appearing cells

FIGURE 4:

Monotonous appearing cells with fine granular chromatin

TABLE 1:

The IHC Panel is tabulated below;

Immunohistochemistry PANEL
• PanCK • Positive

• Beta Catenin • Cytoplasmic Positivity

• Synaptophysin • Negative

• Chromogranin • Negative

• CD56 • Negative

• Inhibin • Negative

• Desmin, Myogenin • Negative

 • Vimentin • Negative

• Germ cell markers Negative

• CEA • Negative

DISCUSSION

Pancreatoblastoma is an uncommon neoplasm that generally occurs in childhood and

demonstrates distinctive histopathological features. Some authors advocate that
imaging findings on ultrasonography (U.S), computed tomography (C.T) and/or
Magnetic Resonance Imaging (MRI) plus an elevated Alpha Feto Protein (AFP) may
suggest correct pre-operative diagnosis. The behavior of PB is unpredictable and its
optimal treatment has not been defined as yet. Complete surgical resection remains
the most commonly associated treatment for long term survival. In unresectable
tumors, responses to pre-operative chemotherapy have been reported by a few
authors.
In patients with local recurrence, spillage or metastasis, adjuvant chemotherapy
and/or radiotherapy should be considered. At present, the prognosis of such cases is
very poor.

The molecular pathogenesis of PB is unknown, but its occasional occurrence in

patients with BWS and the case report of PB associated with Adenomatous Polyposis
may suggest it might bear distinctive molecular genetic alterations.

The criteria for diagnosis of Pancreatoblastoma should include infantile carcinoma of

the pancreas with an encapsulated mass derived from ventral pancreas; histological
identification of epithelial differentiation, including Acinar, glandular or trabecular
architecture, as well as distinct organoid pattern containing globular structures with
elongated cells (squamoid corpuscles); and the presence of Acinar cells with zymogen
granules.

TABLE 2:

Differential diagnosis of pancreatic tumors according with morphological and immunohistochemical criteria
(adapted from Odze and Goldbloom Surgical Pathology of the gastrointestinal tract, 3th edition)

CRITERIA

Pancreati Acinar cell Solid-pseudo Pancreatoblastom

c NET carcinoma papillary a
neoplasm

Nuclei Round and Round to Oval Grooves Variable amounts of Acinar,

oval prominent Uniform squamous, endocrine,
Salt and nucleoli Small nucleoli and primitive component
pepper

Cytoplasm

Scant and wispy Dense Scant

Oncocytic Well defined Tail like
Clear Granular Inclusions

Stroma
Vascular Vascular Mucoid fibro vascular Variable

CK AE1/AE3 Positive Positive Positive Positive

NEC Positive Negative Negative Focal positive

Markers
 Β-catenin Negative Negative Positive Focal positive

Allelic loss on chromosome 11p is the most common genetic alteration in PB, present
in 86%. Loss of Heterozygosis (LOH) of 11p, which includes 11p15 BWS gene and
IGF2, restricted to the cells of the limb showing hypertrophy, has been described in
one BWS-associated PB and a mosaic paternal 11p15 uniparental disomy in the
tumoral cells in another case.

Patients usually present with an upper abdominal mass and either no symptoms or
with nonspecific complaints including epigastric pain (40%), failure to thrive (20%)
and vomiting.
Obstructive jaundice occurs in less than 15% of patients. Diarrhea is noted I many
cases, although it is not clearly related to any hormonal syndrome.
In fact, with the exception of 1 patient who was reported to have had Cushing’s
syndrome and Syndrome of Inappropriate Anti-Diuretic Hormone (SIADH), no
hormone or enzyme secreting Pancreatoblastoma cases have been reported.

TABLE 3:

SYMPTOMS OCCURENCE

Abdominal Mass 40%

Abdominal Pain 40%

Failure to thrive 20%

Obstructive Jaundice 15%

Vomiting 5%

Our patient presented with an abdominal swelling, initially noticed in the upper
abdomen, which gradually increased to occupy the lower abdominal quadrants a well.
He also gave a history of pain in the abdomen and both of these symptoms were from
the last 3 months in duration.

Ultrasound (U.S), CT scan and MRI Scan show variable imaging features, but usually
allow a correct pre-operative diagnosis of PB. Typically, PB is a heterogeneous tumor
with well-defined margins that may appear to arise from any region of pancreas.
Other common findings described by Kohda et al include pancreatic head origin
(44%), pancreatic body and tail origin (56%), hemorrhage (94%), necrosis (90%),
cystic changes (69%) and calcification (48%).

Our patient had

TABLE 4:

Age wise distribution chart of Exocrine Pancreatic Neoplasms

Acinar Cell Carcinoma of the Pancreas Pancreatoblastoma

Typically older patients (40-70 years), rare in children

Predominantly pediatric but 1/3 in adults

Adenosquamous Carcinoma of the Pancreas

Not reported under 20 years of age

Solid Pseudopapillary Neoplasm of the Pancreas

Very rare <5 years

According to the biological growth characteristics of PB and the literature review,

surgery is the treatment of choice. Localized tumor should be completely resected.
This is the most conservative therapy with a good prognosis and no late
complications.

Sometimes, a PancreaticoDeodenectomy, a regional Pancreatectomy or a distal

Pancreatectomy may be necessary.
Our [patient had a localized tumor that was completely resected. No chemotherapy or
radiotherapy was offered. He is doing well currently, and is on a 3 month follow up
free of disease.

Assessment of the prognosis for PB is difficult because of the small number of

reported cases. According to the literature review, long term outcome depends on
complete tumor removal as proposed by Horie et al. or histology of tumor as proposed
by Iseki et al.

CONCLUSION:
 Pancreatoblastoma is a curable tumor. Complete resection is the
treatment of choice.

 However, Apart from surgical resection, optimal treatment has not been
established.

 Chemotherapy and radiotherapy may have a role in recurrent, residual,

unresectable and metastatic disease but the published data are limited.
  In patients with incompletely resected disease, postoperative radiation
may be indicated.

References: