|Year : 2013 | Volume
| Issue : 3 | Page : 537-540
Solid pseudopapillary tumor of pancreas with sickle cell trait: A rare case report
Harish S Permi1, HL Kishan Prasad1, Balakrishna N Shetty2, B Preetham Rai2, U Raghuraja3, Shubha Bhat1
1 Department of Pathology, K S Hegde Medical Academy, Deralakatte, Mangalore, India
2 Department of General Surgery, K S Hegde Medical Academy, Deralakatte, Mangalore, India
3 Department of Radio Diagnosis, K S Hegde Medical Academy, Deralakatte, Mangalore, India
|Date of Web Publication||8-Oct-2013|
H L Kishan Prasad
Associate Professor, Department of Pathology, K S Hegde Medical Academy of Nitte University, Deralakatte, Mangalore
Source of Support: None, Conflict of Interest: None
Solid pseudopapillary tumor of pancreas is a rare pancreatic neoplasm affecting young women, has low malignant potential and amenable for surgical excision with good long-term survival. Sickle cell trait is benign condition, which involves one normal beta-globin chain and one HbS chain. Although it is a benign condition, individuals are prone to have rare complications that may predispose to death under certain circumstances. We report a rare coexistence of solid pseudopapillary tumor of pancreas with sickle cell trait in an 18-year-old female who underwent distal pancreatectomy with splenectomy. Histopathological examination and haemoglobin electrophoresis confirmed the diagnosis.
Keywords: Electrophoresis, pancreas, sickle cell trait, solid pseudopapillary tumor
|How to cite this article:|
Permi HS, Kishan Prasad H L, Shetty BN, Rai B P, Raghuraja U, Bhat S. Solid pseudopapillary tumor of pancreas with sickle cell trait: A rare case report. J Can Res Ther 2013;9:537-40
|How to cite this URL:|
Permi HS, Kishan Prasad H L, Shetty BN, Rai B P, Raghuraja U, Bhat S. Solid pseudopapillary tumor of pancreas with sickle cell trait: A rare case report. J Can Res Ther [serial online] 2013 [cited 2019 Nov 20];9:537-40. Available from: http://www.cancerjournal.net/text.asp?2013/9/3/537/119363
| > Introduction|| |
Solid pseudopapillary tumor (SPT) of the pancreas is a rare cystic pancreatic neoplasm that accounts for only 1-2% of all exocrine pancreatic tumors.  SPT of the pancreas affects predominantly young women, pathogenesis is unclear, and has low malignancy potential. Surgical resection is the main stay of treatment.  Sickle cell trait (SCT) is characterized by the inheritance of a normal haemoglobin gene (HbA) from one parent and an abnormal, mutated sickle haemoglobin gene (HbS), from the other parent.  SCT can occasionally associated with rare but fatal renal medullary cancer, renal papillary necrosis, hyposthenuria, splenic infarction, exertional rhabdomyolysis, and exercise related sudden death.  We report a case of 18-year-old female, who presented with abdominal pain; computed tomography (CT) abdomen revealed a pancreatic mass adherent to spleen. She underwent distal pancreatectomy and splenectomy. Histopathological examination and haemoglobin electrophoresis confirmed the diagnosis SPT and SCT respectively. Postoperative period was uneventful and on regular follow up, she is doing fine. Our case report highlights the rare coexistence of SPT of pancreas and SCT.
| > Case Report|| |
An 18-year-old female presented with dull aching upper abdomen pain since 3 months. There was no history of vomiting, loose stools, constipation, and malena. Systemic examination revealed moderate splenomegaly. Routine haematological, biochemical and serological tests were within normal limits. CT scan abdomen showed a 6.5×7.3×8 cm well defined heterogeneous density solid and cystic lesion in the tail and body of pancreas with specks of calcification and splenomegaly [Figure 1]. Features were suggested of a pancreatic neoplasm. She underwent distal pancreatectomy with splenectomy. Gross showed circumscribed pancreatic mass measuring 10×8×6 cm partly adhering to the spleen. Cut section of mass showed grey white, solid, cystic, hemorrhagic and necrotic areas with small portion of normal pancreatic tissue [Figure 2]. Spleen measured 13×9×6 cm with brownish cut surface. Microscopy of the pancreatic mass revealed; a complex papillary structures with broad fibrovascular cores. The papillary structures were lined by cuboidal to polygonal cells having clear cytoplasm and uniform nucleus [Figure 3]. Focus of calcification, haemorrhage and deposition of cholesterol clefts with giant cells were seen. The blood vessels within the tumor showed sickled RBCs [Figure 4]. Spleen showed features of chronic venous congestion with sinusoids showing sickle cells [Figure 5]. Tumor cells were immunoreactive for vimentin [Figure 6]. Hence final diagnosis of SPT of the pancreas was made. Sickling test and heat solubility tests were inconclusive. Haemoglobin electrophoresis showed Hb A-79%, Hb S-19% and Hb A2-2% confirming the sickle cell trait. Family pedigree analysis was unremarkable. Postoperative period was uneventful. She was given genetic and diet counselling and on regular follow up, she is doing fine.
|Figure 1: Computed Tomography (CT) scan abdomen showed a 6.5×7.3×8 cms well defined heterogeneous density solid and cystic lesion in the tail of pancreas with specks of calcification|
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|Figure 2: Cut section of pancreatic mass showing well circumscribed grey white solid, cystic, hemorrhagic and yellowish necrotic areas|
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|Figure 3: Microscopy from pancreatic mass showing papillary structures lined by cuboidal cells having bland nucleus. (H and E, X 100).|
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|Figure 4: Microscopy from blood vessels within the tumor area-showing sickle shaped red cells. (H and E, X 400)|
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|Figure 5: Microscopy from spleen showing features of chronic venous congestion with sinusoids showing sickle shaped red cells.(H and E, X 400)|
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| > Discussion|| |
The solid pseudopapillary tumor of pancreas was first reported by Frantz in 1959, hence it s also called Frantz tumor. , It is also termed as papillary epithelial neoplasm, papillary cystic carcinoma, solid and cystic tumor of the pancreas and low-grade papillary tumor.  It occurs commonly in young females, between 15 and 35 years. The tumor may occur anywhere in the pancreas but commonly found in the head or the tail of the pancreas.  Abdominal pain and slowly enlarging mass in the upper abdomen are the common symptoms. Some patients are asymptomatic and the mass is detected either during routine examination or after injury.  SPT is detected as a heterogenous mass by ultrasound, CT and magnetic resonance imaging but the findings are not specific. 
The histogenesis of these tumors is unknown but they possibly originate from the primordial cells and lack definite endocrine and exocrine differentiation. ,,, Morphologically SPT is usually encapsulated and is composed of a mixture of solid, cystic, hemorrhagic and necrotic components.  On microscopy, it shows solid and cystic areas with papillary structures lined by cytologically bland cells. Aggregates of foamy histiocytes, cholesterol clefts and cytoplasmic vacuolization can be seen. ,, The tumor cells are immunoreactive for alpha-1-antitrypsin, alpha-1-antichymotrypsin, neuron specific enolase, vimentin and progesterone receptors.  The SPT has to be differentiated from
- Pancreatoblastoma, which shows epithelial elements arranged in well-defined islands separated by stromal bands and solid, hypercellular area composed of nests of polygonal cells with focal acinar differentiation. The presence of squamoid corpuscles is typical for this tumor.
- Ductal adenocarcinoma, which is characterized by tumor cells showing well developed glandular structures, embedded in desmoplastic stroma. 
Surgical excision remains the mainstay of treatment. ,, Spleen preserving distal pancreatectomy is preferable in the setting of malignant neoplasm, because of its putative mechanism for immune surveillance maintenance. Some recommend the splenectomy should be always performed for oncologic reasons when distal pancreatectomy is performed for cancer. , Benoist, analyzed data from 40 patients who underwent distal pancreatectomy for indications other than chronic pancreatitis and found that distal pancreatectomy with splenectomy had a lower morbidity rate, and pancreas-related complications occurred more frequently after spleen-conserving surgery. 
SCT is recognized incidentally in most cases due to absence of symptoms and is present in 8% of African Americans. ,, SCT is associated with venous thromboembolic events, fetal loss, neonatal deaths, preeclampsia, acute chest syndrome, and anemia in pregnancy. There is insufficient evidence to suggest an independent association with retinopathy, cholelithiasis, priapism, leg ulcers, liver necrosis, avascular necrosis of the femoral head and stroke. Despite these associations, the average life span of individuals with sickle cell trait is similar to that of the general population. ,,, Given the large number of people with sickle cell trait, it is important that physicians be aware of these associations.  Our case was, SPT of pancreas where the blood vessels within the tumor area and splenic sinusoids showed sickled RBCs. But there was no haematological abnormality, hence SCT was suspected and haemoglobin electrophoresis confirmed it. In these cases of SCT, genetic and diet counselling is necessary to prevent further complications.
To conclude, our case is a unique rare association of SPT of pancreas with SCT, which merits worth consideration. SCT is a benign condition with various rare complications and coexistence mentioned in the literature, SPT further adds to the list. We stress the need for genetic counselling in these patients, who are diagnosed incidentally to prevent further complications.
| > Acknowledgement|| |
We are grateful to Dr. J. H. Makannavar, Senior Professor and Dr. K. Jayaprakash Shetty, Professor and Head, Department of Pathology, for their valuable help and suggestions.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]