A 27-year old female presented with abdominal pain and vomiting. Physical examination revealed a large abdominal mass. Imaging showed a large heterogeneous mass in the abdomen. The patient underwent surgery where a large solid and cystic mass involving the pancreas and surrounding structures was removed. Histopathology of the mass found it to be a solid pseudopapillary neoplasm of the pancreas, a rare low-grade malignant tumor that predominantly affects young women. The patient recovered well after surgery.
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A Bleeding Abdominal Tumor(Pseudopappilary Pancreatic Tumor)
1. A bleeding tumor in the
abdomen
DR. NASIR MAHMOOD AKRAM (MCPS,FCPS)
SENIOR REGISTRAR GENERAL SURGERY
ARMED FORCES HOSPITAL DHAHRAN
2. Overview:
History
Physical examination
Pre operative management steps
Decision to operate
Intra operative findings
Post operative management
Histopathology
Literature review
Discussion
3. Presenting complaints:
Dizziness
History of fall from a height of about 1 m, hit left half of the body on the ground.
Pain upper abdomen
Vomiting for the last one day.
4. HOPI:
27 years old single Saudi female, presented
in emergency room with one day H/O dizziness resulting in falling down from stairs and
hitting left side of her body on the ground. She suddenly experienced moderate to severe
pain in upper abdomen, radiating to back and aggravated by body moments.
No H/O LOC, fits or respiratory distress.
Patient started to vomit few hours after fall, minimal in amount, non bilious and
multiple times.
She was nauseated most of the time.
No H/O bowel or urinary complaints.
5. Past history:
She is known to have chronic anemia (Hb <10g/dl and lowest one 7.5g/dl) and was
diagnosed as Iron def. anemia.
H/O few visits in ER and at PHCC with complaints of intermittent epigastric pain
,nausea, dizziness and repeated URTI, being treated symptomatically.
In 2014 she had c/o epigastric pain and repeated vomiting and diagnosed as chronic
gastritis (H.Pylori IgG antibody +ve).
No abdominal mass was detected by physical exam, no radiological investigation was
performed.
No H/O surgical intervention.
6. Personal History:
Student, unmarried, no H/O, smoking, addiction or drug abuse.
No H/O DM, HTN or BA.
Regular menstrual cycle.
8. Physical Examination:
A young lady looking anxious, in pain and pale.
Initial Vitals signs were.
Pulse- 106 bpm
Blood Pressure-128/81 mmHg
Temperature-Afebrile
O2 Saturation-96% at room air
The rest of general physical examination was unremarkable.
9. Abdominal Examination
Abdomen, fullness in the upper abdomen.
At upper abdomen, a firm to hard mass, mildly tender occupying epigastrium, left
hypochondrium and reaching below the umbilicus and two fingers above the left iliac
crest.
The surface of the mass was nodular and well demarcated margins and it was slightly
mobile with respiration in cranio caudal direction and dull on percussion.
Bowel sounds. Audible.
Rest of systemic exam was unremarkable.
11. USG Abdomen & Pelvis:
A heterogenous solid mass lesion seen in the epigastrium down to spleen measuring
18.3*12.6 cm.
Liver is normal, no lymphadenopathy or free fluid in abdominal and pelvic cavity.
12.
13. CT scan Abdomen & Pelvis(without contrast)
A huge bossy partially solid mildly hypodense lesion occupying LHCN, epigastrium and
left lumbar region measuring 11.1*14.2*19.9 cm in AP, Transverse and CC dimensions
respectively.
Medial intrinsic calcifications. The surrounding structures including colon , pancreas
and spleen are displaced.
No intra abdominal or pelvic fluid collection.
Impression: Neoplastic lesion of tail of pancreas or GIST.
14.
15.
16.
17.
18. Initial Management:
The resuscitation of patient started in ER, with IV fluids and analgesics with symptomatic
control of presenting complaints.
Patient became stable with BP 130/74, pulse 90 bpm, O2 saturation 100% at 3L/min.
She was transfused 2 units of PRBCs and repeated Hb was 9.97g/dl.
Plan was to do Ct scan abdomen & pelvis with contrast and proceed. Meanwhile patient
developed hypotension and generalized severe abdominal pain & persistent vomiting of
non bilious gastric contents.
Pulse rate was 108bpm and blood pressure dropped to 91/52 mmHg. Abdomen was
distended and tender all over.
Repeat Hb was 9 gm/dl and Wbc 21 K/Ul.
19. Decision was made to proceed with immediate laparotomy.
Abdomen opened through midline incision, due to difficulty in
mobilization of a huge mass transverse extension was given to
left.
20. Intra operative findings:
A huge solid mass with cysts and calcification over the surface covering whole of upper
& left side of abdomen and crossing midline.
Hemoperitoneum of 1.8 L.
Mass was attached to transverse colon, small gut mesentery, hilum of spleen and tail of
pancreas.
Lesser sac was full of blood.
Frozen section of a part of tumor revealed solid pseudopapillary pancreatic tumor.
26. Mass was pushing the surrounding structures but not
infiltrating and was completely excised.
Splenectomy and distal pancreatectomy were performed.
Two drains were placed.
27. Post op recovery:
Post op recovery was smooth, initial 48 hrs in icu.
Post Splenectomy vaccination (Meningococcal & Hib)
was given before discharge.
Post operative Hb was 12g/dl.
She was discharged in a stable condition.
28. Historical Background
Rare Neoplasm
First description by Virginia Frantz in 1959 as
“Papillary cystic tumor of the pancreas”
2-year-old boy who died during an
attempted pancreatic-duodenectomy
1970 Hamoudi et al described the
ultrastructural features of the tumor
Ductal Adenocarcinoma
85%
Others
13%
SPN
2%
Incidence of Pancreatic
Tumors
Ductal
Adenocarcinoma
Others
SPN
29. Historical Background
World Health organization (WHO) classification of pancreatic tumors in 1996 as
‘‘solid pseudopapillary tumor’’ of the pancreas
Different names in the literature:
Papillary epithelial neoplasm of pancreas
Solid and cystic tumor of the pancreas
Adenocarcinoma of pancreas of childhood
Papillary-cystic tumor
solid and papillary epithelial neoplasm
30. SPN
In the current WHO classification, SPN is defined as a
low-grade malignant neoplasm of the exocrine
pancreas. The term SPN gained wide acceptance and is
currently the most frequently used name for this entity.
31. Historical Background
To date, around 700 cases have been reported
More than two-thirds of them in the last 10 years
Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature.
32. SPN
The etiology and the differentiation status of SPN still remained challenging and
enigmatic
The spectrum of cystic and solid and cystic neoplasms of the pancreas is wide and
encompasses at least 14 different tumor types
Cystic lesions and neoplasms of the pancreas. The features are becoming clearer.
33. SPN
The most commonly encountered cystic neoplasms of the pancreas may be
classified into five categories:
Serous microcystic adenoma
Mucinous cystic neoplasms (cystadenoma or cystadenocarcinoma)
Intraductal papillary mucinous neoplasm (IPMN)
Cystic neuroendocrine neoplasms
Solid pseudopapillary neoplasm of the pancreas (SPN).
Among these uncommon pancreatic tumors, SPN represents an exceedingly rare
entity.
34. Epidemiology
SPN usually affects young women at an average age of 28 years
Female: Male ratio of 10:1
20-25% pediatric patients
Sporadic rare cases in men and in the elderly have also been reported
35. Clinical Presentation
Nonspecific presentation
Abdominal discomfort
Abdominal pain
Palpable abdominal mass
Loss of weight
Vomiting
Rarely Obstructive jaundice and Pancreatitis
Slow growth keeps SPN often remains asymptomatic, until the tumor has enlarged
considerably.
Many are detected incidentally on diagnostic imaging for unrelated diseases or after a
blunt abdominal trauma
Surgical management of solid-pseudopapillary neoplasms of the pancreas (Franz or Hamoudi tumors): a large single-
institutional series.
36. Anatomical Site
The most common site tail of the pancreas,
Followed by the head and the body
Unusual presentations
Multicentric tumors in the pancreas
Extra pancreatic sites possibly representing synchronous tumor spread
Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature.
37. Pathogenesis
Postulated to arise from primitive pancreatic cells
From cell lines of the female genital bud
Beta-catenin mutations
Papillary-cystic neoplasm of the pancreas. A sex-steroid dependent tumor.
38. The key histological hallmarks are solid and pseudopapillary proliferation of
homomorphous cells without increased mitoses or cytological atypia
The common expression of progesterone receptor and the strong predilection for
females suggest that it might be a hormone-dependent tumor
Estrogen receptors have not been demonstrated.
Another hypothesis is an extra pancreatic origin from genital ridge anlage-related
cells
39. Histology
Histologically, SPN are commonly well-circumscribed and -encapsulated with
irregular degenerative cystic cavities and hemorrhages.
The tumor contains a mixture of solid, cystic, and pseudopapillary patterns in
various proportions.
The diagnosis can be confirmed by immunohistochemical analysis
40. Diagnosis
Routine laboratory parameters and tumor markers are of no help
Ultrasound and CT/MRI-scan
A large well-circumscribed, heterogeneous mass with varying solid and cystic
components, generally demarcated by a peripheral capsule and occasional calcification.
MRI is superior to CT in distinguishing certain tissue characteristics, such as
hemorrhage, cystic degeneration or the presence of a capsule and may suggest
correct diagnosis
MR imaging features of solid pseudopapillary tumor of the pancreas in adult and pediatric patients.
41. Diagnosis
Angiography usually demonstrates an avascular or hypo vascular tumor and may
help delineate the mass from other involved adjacent structures
The diagnosis can be confirmed by an endoscopic ultrasound scan (EUS) with fine-
needle aspiration (FNA) biopsy or percutaneous core needle biopsy with
ultrasound or CT-guidance
Focal pancreatic lesions: accuracy and complications of US-guided fine-needle aspiration cytology.
42. Differential Diagnosis
Serous microcystic adenoma
Cystadenocarcinoma
Mucinous cystic neoplasms
Cystic neuroendocrine tumors
Cystic acinar cell carcinoma
Teratoma
Pancreatoblastoma
However, the typical constellation of a pancreas-associated solid and cystic upper
abdominal mass with or without calcifications in a young woman should always alert to
the possibility of SPN.
43. General Behaviour and treatment of
Tumor
Low malignant potential with generally excellent prognosis.
Malignant behavior is observed in about 10-15% of the cases
Given their low malignant potential and the excellent overall prognosis, surgical
resection has been the standard of care in the management of SPN
Tumor enucleation and incomplete excision should be avoided due to the risk of
tumor dissemination, development of a pancreatic fistula and the higher
recurrence rate.
Extensive lymphatic dissection or resection of adjacent structures is not warranted
since lymph node metastasis are found in <2%
44. General Behaviour and treatment of
Tumor
Tumor size should not be regarded as a predictor of unresectability because
lesions as large as 30 cm may be resected without problems
Management of a solid pseudopapillary tumor of the pancreas with liver
metastases.Vollmer CM Jr, Dixon E, Grant DR HPB (Oxford). 2003; 5(4):264-7.
Unlike other pancreatic tumors, the stage of the disease does not play any role for
the treatment of SPN
If veins are infiltrated, vascular en-bloc resection and reconstruction with vein
grafts has been proposed and the results were encouraging
Solid-pseudopapillary tumor of the pancreas: a surgical enigma?Martin RC, Klimstra DS,
Brennan MF, Conlon KC Ann Surg Oncol. 2002 Jan-Feb; 9(1):35-40.
45. General Behaviour and treatment of
Tumor
Other therapeutic options such chemotherapy and radiotherapy have been applied in
some cases but they basically have no place with this entity
A role for radiotherapy in the treatment of solid and papillary neoplasms of the
pancreas.Fried P, Cooper J, Balthazar E, Fazzini E, Newall J Cancer. 1985 Dec 15; 56(12):2783-5.
The role of neoadjuvant chemotherapy is not defined
Spleen-preserving distal pancreatectomy following neoadjuvant chemotherapy for papillary
solid and cystic neoplasm of pancreas.Das G, Bhuyan C, Das BK, Sharma JD, Saikia BJ,
Purkystha J Indian J Gastroenterol. 2004 Sep-Oct; 23(5):188-9
Solid-pseudopapillary tumor of the pancreas: a surgical enigma?Martin RC, Klimstra DS,
Brennan MF, Conlon KC Ann Surg Oncol. 2002 Jan-Feb; 9(1):35-40.
46. General Behaviour and treatment of
Tumor
A favorable response to radiotherapy in locally advanced unresectable disease has
also been reported with substantial shrinkage of an unresectable tumor after 6
weeks of radiotherapy
Papillary-cystic neoplasm of the pancreas. A clinicopathologic study concerning the
tumor aging and malignancy of nine cases.Matsunou H, Konishi F Cancer. 1990 Jan 15;
65(2):283-91.
A role for radiotherapy in the treatment of solid and papillary neoplasms of the
pancreas.Fried P, Cooper J, Balthazar E, Fazzini E, Newall J Cancer. 1985 Dec 15;
56(12):2783-5.
47. Prognosis
Overall 5-year survival approaches 97% in patients undergoing surgical resection
Solid-pseudopapillary tumor of the pancreas: a surgical enigma?Martin RC, Klimstra DS,
Brennan MF, Conlon KC Ann Surg Oncol. 2002 Jan-Feb; 9(1):35-40.
Clinically aggressive solid pseudopapillary tumors of the pancreas: a report of two cases
with components of undifferentiated carcinoma and a comparative clinicopathologic
analysis of 34 conventional cases.Tang LH, Aydin H, Brennan MF, Klimstra DS Am J Surg
Pathol. 2005 Apr; 29(4):512-9
48. Prognosis
Death ascribed directly to the tumor is rare and long-term survival (years to
decades) has been described even in the presence of asymptomatic disseminated
disease
Clinically aggressive solid pseudopapillary tumors of the pancreas: a report of two cases
with components of undifferentiated carcinoma and a comparative clinicopathologic
analysis of 34 conventional cases.Tang LH, Aydin H, Brennan MF, Klimstra DS Am J Surg
Pathol. 2005 Apr; 29(4):512-9
49. Prognosis
Clear-cut criteria of malignancy have not been established and it is difficult to
predict the behavior of SPN on histomorphological grounds. The Ki-67 index has
been suggested as a potential indicator of malignant potential and poor outcome
of SPN
Revision 2: an immunohistochemical approach and evaluation of solid pseudopapillary
tumour of the pancreas.Serra S, Chetty R J Clin Pathol. 2008 Nov; 61(11):1153-9.
50. KI-67 Index
Ki-67 is a protein in cells that increases as they prepare to divide into new cells. A
staining process can measure the percentage of tumor cells that are positive for
Ki-67. The more positive cells there are, the more quickly they are dividing and
forming new cells.
51. Prognosis
Features that may indicate an aggressive clinical behavior are
Venous invasion
Diffuse infiltrative growth pattern
Extensive tumor necrosis
Significant nuclear atypia
High mitotic count
Nuclear pleomorphism
Dedifferentiation, DNA aneuploidy, double loss of X chromosomes, trisomy of chromosome 3
and unbalanced translocation between chromosomes 13 and 17
Papillary cystic tumor of the pancreas. A clinicopathologic study of 20 cases with cytologic, immunohistochemical,
ultrastructural, and flow cytometric observations, and a review of the literature.Pettinato G, Manivel JC, Ravetto C,
Terracciano LM, Gould EW, di Tuoro A, Jaszcz W, Albores-Saavedra JAm J Clin Pathol. 1992 Nov; 98(5):478-88.
52. Prognosis
SPN affecting elderly males have been associated with increased likelihood of
malignancy
Solid and pseudopapillary tumor of the pancreas--review and new insights into
pathogenesis.Geers C, Moulin P, Gigot JF, Weynand B, Deprez P, Rahier J, Sempoux C
Am J Surg Pathol. 2006 Oct; 30(10):1243-9.
Solid pseudopapillary neoplasm of the pancreas: distinct patterns of onset, diagnosis,
and prognosis for male versus female patients.Machado MC, Machado MA, Bacchella T,
Jukemura J, Almeida JL, Cunha JE Surgery. 2008 Jan; 143(1):29-34.
53. Case Series
Solid pseudopapillary neoplasm (SPN) of the pancreas: case series and literature
review on an enigmatic entity
By Nikolaos et al (Department of Surgery, University Hospital, Erlangen, Germany
Institute of Pathology, University Hospital, Erlangen, Germany)
54. Case 1
A 23yearold woman presented with upper abdominal swelling for 2 months
presented with worsening abdominal pain
10 cm well defined, nontender, Nonpulsatile mass was palpable in the epigastrium
and right hypochondrium
Apart from a slightly elevated white cell count, haematological and biochemical
studies were unremarkable
Abdominal ultrasound and computed tomography showed a circumscribed
encapsulated heterogeneous mass with solid and cystic areas arising from the
head of pancreas, measuring 12 x 12 x 13 cm. No lymphadenopathy or other
pathological findings were seen
55.
56. USG guided percutaneous core needle biopsy was consistent with SPN of the
pancreas
The Ki67 index was <2%
Laparotomy confirmed a pancreatic head tumor without evidence of
intraabdominal metastasis.
The patient underwent a Whipple operation
57.
58. The resection margins were free of tumor and none of the 11lymph nodes
recovered was positive for malignancy.
On cross section, the tumor measured 16 cm in maximum diameter. It was
composed of cystic and solid components with haemorrhagic areas. The final
histological report confirmed SPN. The patient is currently disease free 24 months
after surgery.
59. Case 2
18 years old girl
20 cm lesion in tail of pancreas
Distal pancreatectomy and splenectomy
Disease free after 25 months
60. Case 3
15yearold girl was initially admitted to a peripheral hospital for acute epigastric pain,
presented after a fall.
MR scan showed an intraabdominal hematoma, involving the pancreas
diagnostic laparoscopy converted to formal laparotomy
local excision (enucleation) of the tumor from the pancreatic head
Five months later, MRI scan demonstrated a 3.5 x 2.4 cm encapsulated cystic and solid
tumor with dilated pancreatic duct
An explorative laparotomy was carried out and a distal pancreatectomy combined with
splenectomy was performed
The patient remains disease free at last followup 51 months after surgery
61. Conclusion
SPN is a rare pancreatic neoplasm of unclear histogenesis that typically affects young
females without significant symptoms.
Appearance on imaging is fairly characteristic and may suggest diagnosis, but in
unclear cases preoperative diagnosis should be accomplished by percutaneous CT-
guided core needle biopsy in order to avoid not indicated preoperative chemotherapy
or radiotherapy.
SPN should be considered in the differential diagnosis of any solid and partly cystic
pancreatic or upper abdominal mass, particularly in young females.
Complete surgical resection of the tumor is the only effective treatment option.
Whenever possible, surgery seems to be justified for local recurrence or metastasis.
