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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 2  |  Issue : 4  |  Page : 93-95

Solid pseudopapillary tumor of head and body of pancreas with pure solid component – A case report with diagnostic dilemma


1 Department of Pathology, Oncquest Laboratories, New Delhi, India
2 Department of Pathology, Bhagwan Mahavir Hospital, Pitam Pura, Delhi, India

Date of Web Publication26-Dec-2018

Correspondence Address:
Dr. Ipsita Dhal
Oncquest Laboratories, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/oji.oji_37_18

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  Abstract 


Solid pseudopapillary tumor (SPT) is a rare neoplasm of the pancreas with malignant potential, predominantly affecting young females. It usually consists of mixed solid and cystic components, and the presence of pure solid component is rarer. It may be locally aggressive, and surgical resection is the treatment of choice with an excellent prognosis. We report a case of SPT of head and body of the pancreas with pure solid component in a 21-year-old female. The patient presented with a chief complaint of severe abdominal pain and vomiting with a palpable mass for 2 years without any past history of abdominal trauma. Contrast-enhanced computed tomography scan showed a well-defined, homogeneously enhancing solid tumor arising from the head and body of the pancreas. With these findings, both benign and low-grade malignant neoplasm of pancreas could be the possible differential diagnoses. Pancreaticoduodenectomy was performed, and the patient was under regular follow-up for the last 28 months without any evidence of the disease.

Keywords: Diagnostic dilemma, pancreas, pure solid component, solid pseudopapillary tumor


How to cite this article:
Dhal I, Saxena S, Swain M. Solid pseudopapillary tumor of head and body of pancreas with pure solid component – A case report with diagnostic dilemma. Oncol J India 2018;2:93-5

How to cite this URL:
Dhal I, Saxena S, Swain M. Solid pseudopapillary tumor of head and body of pancreas with pure solid component – A case report with diagnostic dilemma. Oncol J India [serial online] 2018 [cited 2019 Mar 25];2:93-5. Available from: http://www.ojionline.org/text.asp?2018/2/4/93/248533




  Introduction Top


Solid pseudopapillary tumor (SPT) of pancreas, otherwise known as Frantz's tumor, is a rare pancreatic neoplasm of uncertain lineage consisting of mixed solid and cystic components.[1] The presence of pure solid component is rarer.[2] SPT accounts for 0.2%–2.7% of all pancreatic tumors. It occurs predominantly in young women between second and third decades of life.[3] Most of the cases are benign in nature. Pretreatment diagnosis is difficult due to a paucity of data.[1]

Despite increased recognition, SPT still remains a surgical enigma for the surgeons across the world. Surgery is the treatment of choice, and resection with clear margins is curative. SPT has a good prognosis even in the presence of metastasis.[4] We report a case of SPT of pancreas in a 21-year-old female with review of the literatures.


  Case Report Top


A 21-year-old female presented to our emergency department with the chief complaint of severe abdominal pain and vomiting with a palpable lump per abdomen for 2 years. She had no past history of jaundice or any abdominal trauma. On physical examination, a mass of size approximately 6 cm × 8 cm was palpable at the supraumbilical area, had in consistency and nontender in nature. Contrast-enhanced computed tomography (CECT) scan of the abdomen showed presence of a well-defined, homogeneously enhancing solid tumor of size approximately 8.5 cm × 7.5 cm arising from the head and body of the pancreas [Figure 1]. Tumor markers, such as carcinoembryonic antigen, carbohydrate antigen (CA19-9), and alpha-fetoprotein, were within the normal range. There was no evidence of pancreatic insufficiency or abnormal liver function. Further careful radiological investigation ruled out any presence of distant metastasis. With these findings, a possible diagnosis of benign or low-grade malignant neoplasm was suspected. A gastrointestinal stromal tumor of pancreas, pancreatoblastoma, adenocarcinoma of the pancreas, and other pancreatic tumors could not be ruled out. In view of homogeneously enhancing solid lesion without any further locoregional spread or distant metastasis, an ultrasound guided fine-needle biopsy (FNB) was not advised to avoid needle tract seedling of the disease. The patient was planned for surgery. The patient underwent pancreatoduodenectomy revealing a thick-walled encapsulated mass found in the pancreatic head involving trunk, and intraoperative findings suggested absence of any evidence of locoregional spread. Peritoneal cavity, liver, and spleen were found to be normal. The operated specimen was sent for histopathological analysis. Grossly, the tumor was completely encapsulated, and cut surface revealed solid gray brown tumor with areas of hemorrhage [Figure 2]a and [Figure 2]b. Microscopic examination revealed a well-circumscribed tumor with a fibrous pseudocapsule, composed of sheets of polygonal cells with pseudopapillary formations [Figure 3]a. Individual tumor cells are polygonal with eosinophilic cytoplasm and vesicular nuclei with minimal atypia and occasional mitosis. There was presence of many hyaline globules and sheets of foamy macrophages [Figure 3]b and [Figure 3]c. With the above microscopic findings, a diagnosis of SPT of the pancreas was established. Histopathologically, all the cut margins were clear of the tumor. Further immunohistochemistry analysis revealed diffuse strong positivity for vimentin and cytokeratin, and focal positivity for alpha 1 antitrypsin [Figure 4]a, [Figure 4]b, [Figure 4]c.
Figure 1: Contrast-enhanced computed tomography scan showing the presence of a well-defined, homogeneously enhancing solid tumor of size 8.5 cm × 7.5 cm arising from the head and body of the pancreas

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Figure 2: (a) Gross specimen showing completely encapsulated tumor and (b) the tumor on cut open showing gray-brown surface with areas of hemorrhage

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Figure 3: Microsection section showing (a) arrangement of polygonal tumor cells in sheets and forming pseudorosettes and pseudopapillae, (b) presence of hyaline globules (arrow), and (c) clusters of foamy histiocytes lying in between the tumor cells (arrow) (H and E, ×400)

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Figure 4: Immunohistochemistry analysis showing diffuse, intense positivity for vimentin (a) and cytokeratin (b), and focal expression of alpha-1 antitrypsin (c)

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The postoperative course was uneventful. The patient was on regular follow-up since last 28 months without any evidence of disease.


  Discussion Top


SPT of the pancreas was first described by Virginia Kneeland Frantz in 1959, and in 1996, it was reclassified by the World Health Organization.[5],[6] The present case was reported in a 21-year young female supporting the literature.[1]

Tail of the pancreas followed by head of the pancreas are the most frequent locations for SPT.[3],[4] Extrapancreatic primary for SPT has also been reported such as retroperitoneum, left adrenal gland, and mesentery. Most of the cases are incidentally detected on radiologic examination. Patients usually present with a slowly growing abdominal mass over several years with or without abdominal pain and may be associated with vomiting due to pressure effect of the tumor.[3],[7],[8] Even malignant cases relapse after years of resection.[2] In our case, head and body of the pancreas is the site of location, and presented with abdominal mass with pain and vomiting. These tumors have a quite low MIB-1 index of around 1%. The growth rate can be determined by calculating the doubling time with rare chance due to tumor resection at the time of diagnosis and due to presence of large cystic component not accurately reflecting proliferation.[2]

Pancreatic pseudocyst, hematoma, pancreatoblastoma, or less likely pancreatic adenocarcinoma are considered as differential diagnoses for a large pancreatic SPT. In our case, no past history of trauma and presence of persistent abdominal pain over several years make a hematoma less likely. Also, malignant tumors such as pancreatoblastoma and adenocarcinoma are less likely in the presence of relative stability of patients over several years. Our patient without a history of pancreatitis is less likely to suggest that the lesion was a pseudocyst.[1]

Imaging features for SPT is nonspecific. Sonographically, these tumors are large heterogeneous solid cystic masses with a thick capsule and peripheral calcifications. Whereas on computed tomography, most of the tumors are well-defined solid cystic masses with calcifications.[1] However, we found only solid component of the tumor on CECT scan, which is rare.[2] According to Suzuki et al., radiological findings for SPT showed calcification in 39.4% of cases, capsule formation in 51.5%, cystic components in 69.7%, solid components in 93.9%, and internal bleeding in 36.4% of cases.[9] The tumor is usually appear as hypointense mass on T1-weighted magnetic resonance imaging with areas of increased signal corresponding to hemorrhage and necrosis and with decreased signal corresponding to a fibrous capsule. On T2 sequences, these tumors have heterogeneous signal with enhancement on postcontrast sequences.[1] Blood tests are usually within normal range, and there is no specific tumor marker to be elevated for SPT of the pancreas as in our case.[3],[8]

Gross picture of SPT is usually revealed a well-demarcated lesion with a rim of fibrous capsule and can be either totally cystic or partially cystic in 92%, while tumors with a pure solid component is rare.[2],[4] Invasion of adjacent organs such as spleen and duodenum is rare. On cut sections, SPT shows solid and yellow areas with cystic, necrotic, and hemorrhagic zones.[4] We found completely encapsulated tumor on gross examination, and cut surface revealed solid gray brown tumor with areas of hemorrhage without any cystic areas.

Microscopically, SPT shows presence of solid and pseudopapillary structures, intensive vascularization, or hemorrhagic pseudocystic structures in various proportions. According to few authors, preoperative FNB may distinguish benign lesion from malignant lesion. However, other authors suggest uncertainty of FNB in diagnosis with possible tumor cell spread, and FNB was not performed in our case to avoid tumor seedling.[7]

Histopathologic criteria of malignancy are not well established for SPT of the pancreas. However, factors predicting aggressive behavior for the tumor are size >5 cm, nuclear atypia, vascular and perineural invasion, invasion of surrounding tissues, and high proliferative index.[10] Immunochemistry profile may reveal positivity for alpha-1 antitrypsin (>90%), vimentin (>90%), neuron-specific enolase (>80%), chromogranin A, cytokeratin, synaptophysin, progesterone, and estrogen receptors.[3] In our case, alpha-1 antitrypsin was focally positive, and vimenti and cytokeratin were strongly positive, supporting literature.

Overall survival rate of these patients is nearly 95%. Radical resection is commonly performed. Pancreatoduodenectomy is done for pancreatic head tumor as in our case and a distal pancreatectomy with or without splenectomy for pancreatic body or tail tumor. Local resection or enucleation is the pancreatic parenchyma-sparing operation and can be an option if a curative resection with free margin is achievable.[3],[8] The presence of metastasis or vascular invasion is not a contraindication for surgical resection. Follow-up is important due to the potential of local recurrence and distant metastasis.[8]

In summary, SPT with pure solid component is rare. Preoperative diagnosis is difficult due to nonspecific clinical and radiological features. In spite of multiple differential diagnoses, preoperative FNB is not advisable to avoid needle tract seedling of disease. Intraoperative frozen section can be performed for optimal management of the cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Utturkar A, Yu Z, Lawrence C. Solid pseudopapillary tumor of the pancreas: A difficult presentation of an uncommon tumor. Radiol Case Rep 2018;13:1104-7.  Back to cited text no. 1
    
2.
Kato T, Egawa N, Kamisawa T, Tu Y, Sanaka M, Sakaki N, et al. A case of solid pseudopapillary neoplasm of the pancreas and tumor doubling time. Pancreatology 2002;2:495-8.  Back to cited text no. 2
    
3.
Branco C, Vilaça S, Falcão J. Solid pseudopapillary neoplasm – Case report of a rare pancreatic tumor. Int J Surg Case Rep 2017;33:148-50.  Back to cited text no. 3
    
4.
Lanke G, Ali FS, Lee JH. Clinical update on the management of pseudopapillary tumor of pancreas. World J Gastrointest Endosc 2018;10:145-55.  Back to cited text no. 4
    
5.
Frantz VK. Tumors of the pancreas. In: Bumberg CW, editor. Atlas of Tumor Pathology. VII. Fascicles 27 and 28. Washington: Armed Forced Institute of Pathology; 1959. p. 32-3.  Back to cited text no. 5
    
6.
Kloppel G, Solcia E, Longnecker DS, Capella C, Sobin LH; World Health Organization. World Health Organization International Histological Classification of Tumours. 2nd ed. Berlin: Springer; 1996.  Back to cited text no. 6
    
7.
Słowik-Moczydłowska Ż, Gogolewski M, Yaqoub S, Piotrowska A, Kamiński A. Solid pseudopapillary tumor of the pancreas (Frantz's tumor): Two case reports and a review of the literature. J Med Case Rep 2015;9:268.  Back to cited text no. 7
    
8.
Guo N, Zhou QB, Chen RF, Zou SQ, Li ZH, Lin Q, et al. Diagnosis and surgical treatment of solid pseudopapillary neoplasm of the pancreas: Analysis of 24 cases. Can J Surg 2011;54:368-74.  Back to cited text no. 8
    
9.
Suzuki S, Hatori T, Furukawa T, Shiratori K, Yamamoto M. Clinical and pathological features of solid pseudopapillary neoplasms of the pancreas at a single institution. Dig Surg 2014;31:143-50.  Back to cited text no. 9
    
10.
Lakhtakia R, Al-Wahaibi K, Zahid KF, Malik KA, Burney IA. Solid pseudopapillary neoplasm of the pancreas: A case report with review of the diagnostic dilemmas and tumor behavior. Oman Med J 2013;28:441-4.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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