LETTERS TO THE EDITOR

 

Solid pseudopapillary neoplasm of the pancreas or Frantz's tumour: Report of two cases with different locations

Neoplasia sólido pseudopapilar de páncreas o tumor de Frantz: presentación de dos casos con diferente ubicación

 

 

---

Key words: Pancreas tumour. Solid pseudopapillary tumour of the pancreas. Diagnosis. Treatment.

Palabras clave: Neoplasia de páncreas. Tumor sólido-pseudopapilar. Diagnóstico. Tratamiento.

---

 

 

Dear Editor,

Solid pseudopapillary tumour of the pancreas (SPTP) was described by Frantz in 1959 (1). It is an uncommon neoplasm with an incidence estimated at between 0.2 and 2.7 % of all pancreatic tumours, although it may account for 50% in paediatric patients.

 

Case reports

We report 2 cases in women aged 30 and 27 years, who are currently under follow-up. Both presented with non-specific clinical features such as epigastric pain, vomiting, diarrhoea, fever or weight loss over the previous months. Ultrasound disclosed heterogeneous masses in the tail and head of the pancreas, respectively. The study was completed in both women with computed tomography and magnetic resonance, which revealed heterogeneous lesions with solid and cystic components. They underwent surgery with a preoperative suspicion of SPTP: Distal splenopancreatectomy in the patient with the tumour in the tail of the pancreas and cephalic pancreatoduodenectomy with pylorus conservation in the woman with the tumour in the head of the pancreas. In both women, the pathological anatomy and immunohistochemistry, with positivity for CD10, CD56, vimentin, neuronal enolase and progesterone receptors, and mild positivity to chromogranin, established a final diagnosis of SPTP.

SPTP occurs 90-95 % of the time in young women in their 3^rd decade of life (2) and has a preference for Asians (3). The most common location is the body and tail and on rare occasions it is found in the retroperitoneum, ectopic pancreatic tissue, mesentery or liver (4). Its growth is slow, which is why patients are asymptomatic (5) or have non-specific clinical features and why diagnosis is usually casual in 15 % of cases (6). Imaging tests are usually typical: well-delimited heterogeneous mass due to its haemorrhagic necrotic solid and cystic components (7). Fine-needle puncture-aspiration is controversial, as there is no clear efficiency for differential diagnosis and it may be confused with a pseudocyst when only necrotic material is collected. The origin of SPTP is still uncertain, although a theory gaining strength is that it originates in pluripotential pancreatic cells which would be favoured by genetic factors, and hormone stimuli due to a greater frequency in women and the presence of hormone receptors (8). Immunohistochemistry usually shows a positive reaction to neuron-specific enolase, vimentin, alpha1-antitripsin, somatostatin, glucagon or insulin.

Depending on the location of the SPTP a type of surgery must be performed (distal or cephalic excision) with complete resection in order to obtain a good prognosis and a low rate of local recurrence and metastases; the 10-year survival rate is good and a long-term follow-up is recommended (9). When the lesion is superficial or complete resection is not possible, an enucleation or partial resection can be performed with acceptable outcomes. No clear indication for treatment with radiation therapy or chemotherapy has been shown. Metastases are found at the time of diagnosis in 10-15 % of patients, especially in the liver and usually as the only metastasis (10). In these cases of local recurrence or metastases, the surgical approach may not be recommended due to the long survival rate.

Sources of funding: Article funded in part by the FFIS (Foundation for Health Research and Training in the Region of Murcia, Spain, Group FFIS-008) and the University of Murcia (Spain).

 

María Ángeles Verdú-Fernández, María Luisa García-García,
María Pilar Guillén-Paredes, Juan Gervasio Martín-Lorenzo and José Luis Aguayo-Albasini
Department of General and Digestive Surgery. Hospital Universitario Morales Meseguer.
Campus de Excelencia Internacional "Mare Nostrum". Universidad de Murcia. Murcia, Spain

 

References

1. Frantz VK. Papillary tumors of the pancreas: Benign or malignant tumors of the pancreas. In: Frantz UK, editor. Atlas of tumor pathology. 1st series, Fascicles 27 and 28. Washington DC: Armed Forces Institute of Pathology; 1959. p. 32-3.         [ Links ]

2. Navarro S, Ferrer J, Bombí JA, López-Boado MA, Ayuso JR, Ginés A, et al. Neoplasia sólida seudopapilar de páncreas. Estudio de 6 casos. Med Clin 2012;138(3):114-8.         [ Links ]

3. Iribarren M, de Castro G, Fernández R, Meléndez R, Freiria G, García F, et al. Neoplasia epitelial pseudopapilar sólido-quística de páncreas (tumor de Frantz). Estudio de dos nuevos casos. Rev Esp Enferm Dig 2004;96:877-8.         [ Links ]

4. Hidalgo PM, Ferrero HE. Neoplasia epitelial sólida quística y papilar del páncreas. Un reto de diagnóstico y tratamiento. Rev Esp Enferm Dig 2006;98:801-8.         [ Links ]

5. Durán I, Docobo F, Alamo JM, Trabado P. Tumor de Frantz en mujer de 57 años. Rev Esp Enferm Dig 2003;95:591.         [ Links ]

6. Papavramidis T, Papavramidis S. Solid pseudopapillary tumors of the pancreas: Review of 718 patients reported in English literature. J Am Coll Surg 2005;200:965-72.         [ Links ]

7. A-Cienfuegos J, Lozano MD, Rotellar F, Martí P, Pedano N, Arredondo J, et al. Solid pseudopapillary tumor of the pancreas (SPPT). Still an unsolved enigma. Rev Esp Enferm Dig 2010;102:722-8.         [ Links ]

8. Frago R, Fabregat J, Jorba R, García-Borobia F, Altet J, Serrano MT, et al. Solid pseudopapillary tumors of the pancreas: diagnosis and curative treatment. Rev Esp Enferm Dig 2006;98:809-16.         [ Links ]

9. Butte JM, Brennan MF, Gönen M, Tang LH, D'Angelica MI, Fong Y, et al. Solid pseudopapillary tumors of the pancreas. Clinical features, surgical outcomes, and long-term survival in 45 consecutive patients from a single center. J Gastrointest Sug 2011;15:350-7.         [ Links ]

10. Yu PF, Hu ZH, Wang XB, Guo JM, Cheng XD, Zhang YL, et al. Solid psedopapillary tumor of the pancreas: A review of 553 cases en Chinese literature. World J Gastroenterol 2010;16:1209-14.         [ Links ]