Albany Medical Review

Case Review: A 39-year-old Asian Female with Abdominal Pain

Tanya Needham, MD

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A 39-year-old Asian female presented to the emergency department with a one year history of dull epigastric pain. The patient reported that the abdominal pain radiated to the left upper quadrant. She characterized the pain as a constant dull ache, occasionally sharp, and without any obvious aggravating or alleviating factors. She also reported early satiety for approximately one month. Neither the satiety nor the pain was relieved by defecation. She denied any nausea, vomiting, diarrhea, change in bowel movements, trauma, fever, chills, or weight loss. 

The patient had no significant past medical or surgical history other than three normal vaginal deliveries.  Her only home medication was a multivitamin with no known drug allergies. She moved to the United States from Taiwan two years ago, lives with her husband and three children and is employed at a local Chinese restaurant. Family history was noncontributory, without history of cancer, gastrointestinal disorders, or hepatitis.

On presenting physical examination, the patient was afebrile, blood pressure was 114/77 mmHg, heart rate was 72 bpm, respirations were 16 bpm, with a pulse oximetry of 97% on room air, and in no apparent distress. On inspection, she had anicteric sclerae and moist oral mucosa without lesions. There was no neck, axillary, or inguinal lymphadenopathy. Heart and lung exam were unremarkable. Her abdomen was soft, nondistended with normoactive bowel sounds in all four quadrants. There was minimal tenderness in the left upper quadrant and midepigastic region with deep palpation but no guarding or rebound tenderness. There were no palpable masses, hepatosplenomegaly, or fluid wave appreciated. Skin exam was normal without any rashes, jaundice, or ecchymoses. Neurological exam was normal without focal deficits.

Based on her symptoms and physical examination the differential diagnoses included:  gastritis, GERD, peptic ulcers, hepatitis, pancreatitis, or malignancy. Due to the indolent nature and nonspecificity of her presenting symptoms, malignancy was high on the differential diagnosis. Laboratory results are noted in Table 1.

 

Table 1. Admission Laboratory Data

 *Note: (H) indicates high.

The only abnormal laboratory data was a mildly elevated total bilirubin, which may be due to hepatocellular diseases, impaired excretion, biliary obstruction, overproduction of bilirubin, or impaired uptake by the liver.  Pancreatitis was ruled out due to normal levels of amylase and lipase. Additionally, hepatitis was unlikely in the setting of normal liver function tests.

The abdominal radiograph performed demonstrated a normal study without bowel obstruction or masses. Computerized tomography of the abdomen and pelvis revealed a heterogenous 8x6.3x6 cm tumor of the pancreatic tail with central necrosis, multiple enhancing liver lesions suspicious for metastatic disease, and fatty infiltration of the liver (See Figure 1).

Figure 1. Computerized Tomography of the Abdomen/Pelvis

Due to these suspicious findings, the gastroenterology service was consulted, for further evaluation for malignancy.  On Day three of her hospital course, an endoscopic ultrasound was performed to further evaluate the pancreatic tumor. The endoscopic ultrasound revealed a normal stomach and duodenum with a pancreatic duct with mildly irregular contoured appearance measuring 2.4mm in diameter and with hyperechoic walls. An irregular large mass was identified in the pancreatic tail measuring 73.6mm by 64.8mm and it was noted to be heterogeneous, lobulated, septated, and mixed solid and cystic. A fine needle aspirate of the pancreatic mass revealed cytologic features consistent with a neoplastic process with the differential diagnosis including cystic pancreatic neoplasm and solid pseudopapillary tumor. Carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA-19-9) were drawn and revealed normal levels of 0.1 ng/ml and 3.1 respectively. 

Surgical oncology was then consulted to further evaluate and manage this pancreatic tail mass.  The patient underwent a subtotal pancreatectomy with splenectomy and liver wedge biopsy. The surgical pathology revealed a 9cm solid pseudopapillary tumor of the pancreas with tumor focally extending into the peripancreatic soft tissue but with clear margins. There was no vascular invasion identified or tumor present in fifteen peripancreatic lymph nodes. The liver was found to have mild non-specific chronic hepatitis without necrosis, no bridging fibrosis, and no tumor identified. The spleen was unremarkable without tumor present. The patient had an uneventful post-operative course and was subsequently discharged.

 

Discussion

This tumor was first described in 1959 by Frantz and it is often referred to as a Frantz tumor, named after the author who first described it.¹ It may also be labeled as a solid cystic tumor, papillary epithelial neoplasia, solid and papillary epithelial neoplasia, or papillary epithelial tumor.¹ In 1996, this tumor was included in the WHO classification of pancreatic tumors.²    

Solid pseudopapillary tumors of the pancreas represent a rare diagnosis in the differential diagnosis of abdominal pain. They comprise of 0.3% to 2.7% of all pancreatic tumors.¹ The origin of the solid pseudopapillary tumor has not yet been identified. It is thought to originate either from ductal epithelium, acinar cells, or from endocrine cells.³ Another belief is that this tumor arises from pluripotent embryonic cells of the pancreas, which were attached to the pancreatic tissue during early embryogenesis.³

Young women are predominantly affected by solid pseudopapillary tumors with a male to female ratio  1:9.5.³  The average age of presentation is 35 but can range between 8 to 67 years old. There are no specific risk factors and the etiology is unknown.

These tumors are generally benign, but can progress into a low grade malignancy. Pseudopapillary tumors can arise from any portion of the pancreas but the most common location of the tumor is the tail of the pancreas (35.9%). The head is the second most common location (34%).⁴

The incidence of malignancy among solid pseudopapillary tumors is approximately 14.7%. Malignant potential is generally associated with features such as advanced age, cellular atypia, vascular invasion, perineural invasion, and invasion of adjacent organs.⁵ The most common sites of metastasis are the liver (42%), peritoneum (42%), and lymph nodes (25%).³

The symptoms of this tumor include abdominal fullness or discomfort. There have been isolated case reports of presentation with abdominal pain secondary to rupture of the tumor.³ The usual presentation of the tumor is incidentally either on physical examination or during imaging studies performed for unrelated reasons. Radiographically, (as seen in figure 1) the solid pseudopapillary tumor of the pancreas is a well circumscribed heterogeneous mass with variable amounts solid and cystic components. The tumor can be located anywhere along the pancreas, but there have been reports of the tumor arising in the mesocolon or retroperitoneum.¹ These locations have been presumed from an ectopic location of the pancreas.

Solid pseudopapillary tumors average 8 to 10cm in diameter with larger tumors having a more cystic component. These tumors typically have a fibrous capsule that is composed of compressed pancreas parenchyma.

Computerized tomorgraphy is approximately 60% sensitive in the diagnosis of cystic pancreatic masses. Magnetic resonance imaging (MRI) is known to distinguish internal tissue characteristics of the tumors better than CT and provides greater information about respectability. Recently, endoscopic ultrasound scan with fine-needle aspiration biopsy has become more popular diagnostic modality and can be useful in preoperatively identifying the tumor.⁴

The treatment for solid pseudopapillary tumors is wide local excision with clear margins. This is usually adequate to prevent recurrence and the prognosis of these tumors is excellent.   Complete surgical resection has been reported to provide more than a 95% cure rate. The tumors may metastasize to the liver and peritoneum, but metastasis to the lymph nodes is uncommon.³ Patients with local recurrence or metastasis can experience long-term survival after surgical re-excision.

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References

1. Eder F, Schulz HU, Röcken C, Lippert H. Solid-pseudopapillary tumor of the pancreatic tail. World J Gastroenterol  2005; 11(26): 4117-4119
2. Klöppel G, Solcia E, Longnecker DS, Capella C, Sobin LH. Histological typing of tumours of the exocrinic  pancreas. In: WHO International Classification of Tumours. Berlin Heidelberg New York, Springer 1996: 8452/1
3. Mao, C, Guvendi, M, Domenico, DR, et al. Papillary cystic and solid tumors of the pancreas: A pancreatic embryonic tumor? Studies of three cases and cumulative review of the world's literature. Surgery 1995; 118:821.
4. Kang CM, Kim KS, Choi JS, Kim H, Lee WJ, Kim BR.  Solid pseudopapillary tumor of the pancreas suggesting malignant potential. Pancreas. 2006 Apr: 32 (3): 276-80.
5. Nishihara K, Nagoshi M, Tsuneyoshi M, et al. Solid pseudopapillary cystic tumors of the pancreas. Assessment of their malignant potential. Cancer. 1993;71:82-92.

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