Pseudopapillary cystic neoplasms (also known as cystic-solid, solid papillary neoplasms, or Franz’s tumor) are a low-grade malignant neoplasms occurring predominantly in young women (>90%) between 30 and 40 years of age. Among the cystic neoplasms, pseudopapillry neoplasms of the pancreas are the least common. According to the WHO classification of cystic tumors, their origin is uncertain, and the biologic behavior is poorly defined. The published case series indicate that pseudopapillary cystic neoplasms are slow-growing, low-aggressive tumors, and even when malignant usually have a favorable prognosis. 10–15% of all patients will have metastases, which are frequently present at the time of first diagnosis.

Small pseudopapillary cystic neoplasms are mostly diagnosed incidentally. When the lesion grows large enough to cause symptoms, abdominal pain is the predominant and, sometimes, the only symptom present. The pain may be associated with a palpable abdominal mass, anorexia, or weight loss, but any of these signs may occur in isolation. These patients usually complain of a full sensation and abdominal discomfort, and only on examination a mass be appreciated, especially in the left upper quadrant.

On cross-sectional imaging, cystic pseudopapillary neoplasms are well-vascularized and encapsulated masses with definite margins. Calcifications and septa may be seen inside the mass, but they are not pathognomonic. Instead, the distinctive findings of these tumors are the alternation of solid and cystic areas, in which a necrotic hemorrhagic component may be present. These findings may be seen in the same lesion, possibly with differences in the proportions of the two components. Endoscopic ultrasound with fine-needle biopsy can help to confirm the diagnosis.

Surgical treatment must be considered in all the patients diagnosed with cystic pseudopapillary neoplasms, based on the still unknown biological behavior and potential malignancy of these tumors. The laparoscopic approach has been shown to be safe and feasible, if expertise is available. Metastatic disease is not considered a contraindication to surgery, as survival after the resection of liver metastases is favorable. Recurrences are seen mainly in malignant cystic pseudopapillary neoplasms, but the long-term survival of these patients has been reported if they are treated. 

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Papavramidis T, et al. J Am Coll Surg. 2005;200:965-972.