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Solid pseudopapillary tumor of the pancreas diagnosed postpartum
Solid pseudopapillary tumor of the pancreas diagnosed postpartum Sergio Huerta, MD, Mark Janssen, MD, and Daniel R. Marcus, MD, Anaheim, Calif
From the Department of Surgery, University of California, Irvine, Medical Center, Kaiser Permanente
CASE REPORT A 29-YEAR-OLD HISPANIC WOMAN was referred to the surgical service for management of a large intraabdominal mass. She initially presented to her primary care physician with complaints of abdominal discomfort, fullness in the left upper quadrant, and inability to lose weight 3 months’ postpartum. She had no systemic symptoms. Her past medical history, review of systems, and a family history were otherwise unremarkable. Physical examination demonstrated moderate abdominal distention and a distinct mass in the left upper quadrant. She had no abdominal tenderness. A computed tomography scan of the abdomen revealed a well-encapsulated, solid, homogeneous 9.6- 3 12- 3 14-cm intra-abdominal mass in the left upper quadrant, suggesting a pancreatic origin (Fig 1). The liver, spleen, and adrenals were radiographically normal. Tumor markers, including alpha-fetoprotein, carcinoembryonic antigen (CEA), cancer antigen (CA)125, and human chorionic gonadotropin (hCG) were within normal levels. At laparotomy, she had a large, well-circumscribed, 781-g mass in the left upper quadrant arising from the tail of the pancreas. The mass was dissected off the transverse mesocolon, preserving the vascular supply of the bowel and integrity of the capsule of the mass. A distal pancreatectomy was then performed with a gastrointestinal anastomosis device. Pathologic examination demonstrated a lobulated tumor with brown solid areas, admixed with zones of hemorrhage (Fig 2). Immunohistochemical stains for cytokeratin, s100, synaptophysin, and chromogranin were negative. Tumor cells were strongly positive for vimentin (Fig 3, A). Additionally, patchy
Fig 1. Computed tomography of the abdomen with oral and intravenous contrast demonstrates a solid mass in the left upper abdomen.
Fig 2. Gross tumor demonstrates solid and cystic areas with zones of hemorrhage.
Accepted for publication December 3, 2003. Reprint requests: Sergio Huerta, MD, 1415 W. North Avenue #202, Anaheim, CA 92801. E-mail: [email protected]. Surgery 2005;137:480-1. 0039-6060/$ - see front matter Ó 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.surg.2003.12.018
480 SURGERY
staining for alpha-1-antitrypsin was noted (Fig 3, B). This histologic analysis was consistent with solid pseudopapillary tumor of the pancreas. The patient had an uneventful intraoperative and postoperative course. She was discharged home on postoperative day 4, and was doing well at a 4-week follow-up visit.
Huerta, Janssen, and Marcus 481
Surgery Volume 137, Number 4
Fig 3. A, Immunohistochemical stain of the tumor demonstrates tumor cells strongly positive for vimentin. B, Immunohistochemical stain of the tumor demonstrates patchy areas of staining for alpha-1-antitripsin.
DISCUSSION Solid pseudopapillary tumors of the pancreas (SPTP) are rare, benign, or low-grade malignant epithelial tumors.1 The low 1% to 2% frequency of SPTP among exocrine tumors appears to have risen in recent years.2 The signs and symptoms of SPTPs are related to their mass effect, and consist of abdominal discomfort or pain. When these tumors present with symptoms, physical examination demonstrates an easily palpable, well-demarcated mass in the abdomen. These are generally large tumors with an average size of 8 to 10 cm.3 Serologic tumor markers and laboratory values are usually within normal limits. Computed tomography is the test of choice and demonstrates a welldefined, solid, and cystic mass. The most useful histochemical markers are alpha-1-antitrypsin, alpha-1-antichymotrypsin, neuron-specific enolase, and vimentin.1 Prognosis for patients with SPTP is excellent. Surgical resection is the mainstay of treatment. SPTPs are tumors with unknown etiology but clear biology. Even though these tumors can reach sizes up to 18 cm, they rarely present with metastatic disease.1 Additionally, tumor size is not related to aggressive behavior.3 No environmental, genetic, or metabolic abnormalities have been found to be associated with SPTP. However, the current number of case reports of SPTPs in the lit-
erature strongly suggests a hormonal component to their pathogenesis.3 First, these tumors affect women in over 90% of the cases. Second, most women are affected during childbearing age, with an average age of 27 years old.3-5 In addition to these observations, as described in the present case, SPTP was diagnosed postpartum. Although this case does not establish a causal linkage, together with the typical presentation of over 500 cases reported in the literature, a hormonal component in the pathogenesis of SPTP should be suggested.
REFERENCES 1. Pettinato G, Di Vizio D, Manivel JC, Pambuccian SE, Somma P, Insabato L. Solid-pseudopapillary tumor of the pancreas: a neoplasm with distinct and highly characteristic cytological features. Diagn Cytopathol 2002;27:325-34. 2. Klimstra DS, Wenig BM, Heffess CS. Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. Semin Diagn Pathol 2000;17:66-80. 3. Solcia E, Capella C, Kloppel G. Tumors of the pancreas. Atlas of tumor pathology. Washington, DC: Armed Forces Institute of Pathology; 1997. p. 120-44. 4. Bombi JA, Milla A, Badal JM, Piulachs J, Estape J, Cardesa A. Papillary-cystic neoplasm of the pancreas. Report of two cases and review of the literature. Cancer 1984;54:780-4. 5. Sanchez JA, Newman KD, Eichelberger MR, Nauta RJ. The papillary-cystic neoplasm of the pancreas. An increasingly recognized clinicopathologic entity. Arch Surg 1990;125: 1502-5.
From the Department of Surgery, University of California, Irvine, Medical Center, Kaiser Permanente
CASE REPORT A 29-YEAR-OLD HISPANIC WOMAN was referred to the surgical service for management of a large intraabdominal mass. She initially presented to her primary care physician with complaints of abdominal discomfort, fullness in the left upper quadrant, and inability to lose weight 3 months’ postpartum. She had no systemic symptoms. Her past medical history, review of systems, and a family history were otherwise unremarkable. Physical examination demonstrated moderate abdominal distention and a distinct mass in the left upper quadrant. She had no abdominal tenderness. A computed tomography scan of the abdomen revealed a well-encapsulated, solid, homogeneous 9.6- 3 12- 3 14-cm intra-abdominal mass in the left upper quadrant, suggesting a pancreatic origin (Fig 1). The liver, spleen, and adrenals were radiographically normal. Tumor markers, including alpha-fetoprotein, carcinoembryonic antigen (CEA), cancer antigen (CA)125, and human chorionic gonadotropin (hCG) were within normal levels. At laparotomy, she had a large, well-circumscribed, 781-g mass in the left upper quadrant arising from the tail of the pancreas. The mass was dissected off the transverse mesocolon, preserving the vascular supply of the bowel and integrity of the capsule of the mass. A distal pancreatectomy was then performed with a gastrointestinal anastomosis device. Pathologic examination demonstrated a lobulated tumor with brown solid areas, admixed with zones of hemorrhage (Fig 2). Immunohistochemical stains for cytokeratin, s100, synaptophysin, and chromogranin were negative. Tumor cells were strongly positive for vimentin (Fig 3, A). Additionally, patchy
Fig 1. Computed tomography of the abdomen with oral and intravenous contrast demonstrates a solid mass in the left upper abdomen.
Fig 2. Gross tumor demonstrates solid and cystic areas with zones of hemorrhage.
Accepted for publication December 3, 2003. Reprint requests: Sergio Huerta, MD, 1415 W. North Avenue #202, Anaheim, CA 92801. E-mail: [email protected]. Surgery 2005;137:480-1. 0039-6060/$ - see front matter Ó 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.surg.2003.12.018
480 SURGERY
staining for alpha-1-antitrypsin was noted (Fig 3, B). This histologic analysis was consistent with solid pseudopapillary tumor of the pancreas. The patient had an uneventful intraoperative and postoperative course. She was discharged home on postoperative day 4, and was doing well at a 4-week follow-up visit.
Huerta, Janssen, and Marcus 481
Surgery Volume 137, Number 4
Fig 3. A, Immunohistochemical stain of the tumor demonstrates tumor cells strongly positive for vimentin. B, Immunohistochemical stain of the tumor demonstrates patchy areas of staining for alpha-1-antitripsin.
DISCUSSION Solid pseudopapillary tumors of the pancreas (SPTP) are rare, benign, or low-grade malignant epithelial tumors.1 The low 1% to 2% frequency of SPTP among exocrine tumors appears to have risen in recent years.2 The signs and symptoms of SPTPs are related to their mass effect, and consist of abdominal discomfort or pain. When these tumors present with symptoms, physical examination demonstrates an easily palpable, well-demarcated mass in the abdomen. These are generally large tumors with an average size of 8 to 10 cm.3 Serologic tumor markers and laboratory values are usually within normal limits. Computed tomography is the test of choice and demonstrates a welldefined, solid, and cystic mass. The most useful histochemical markers are alpha-1-antitrypsin, alpha-1-antichymotrypsin, neuron-specific enolase, and vimentin.1 Prognosis for patients with SPTP is excellent. Surgical resection is the mainstay of treatment. SPTPs are tumors with unknown etiology but clear biology. Even though these tumors can reach sizes up to 18 cm, they rarely present with metastatic disease.1 Additionally, tumor size is not related to aggressive behavior.3 No environmental, genetic, or metabolic abnormalities have been found to be associated with SPTP. However, the current number of case reports of SPTPs in the lit-
erature strongly suggests a hormonal component to their pathogenesis.3 First, these tumors affect women in over 90% of the cases. Second, most women are affected during childbearing age, with an average age of 27 years old.3-5 In addition to these observations, as described in the present case, SPTP was diagnosed postpartum. Although this case does not establish a causal linkage, together with the typical presentation of over 500 cases reported in the literature, a hormonal component in the pathogenesis of SPTP should be suggested.
REFERENCES 1. Pettinato G, Di Vizio D, Manivel JC, Pambuccian SE, Somma P, Insabato L. Solid-pseudopapillary tumor of the pancreas: a neoplasm with distinct and highly characteristic cytological features. Diagn Cytopathol 2002;27:325-34. 2. Klimstra DS, Wenig BM, Heffess CS. Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. Semin Diagn Pathol 2000;17:66-80. 3. Solcia E, Capella C, Kloppel G. Tumors of the pancreas. Atlas of tumor pathology. Washington, DC: Armed Forces Institute of Pathology; 1997. p. 120-44. 4. Bombi JA, Milla A, Badal JM, Piulachs J, Estape J, Cardesa A. Papillary-cystic neoplasm of the pancreas. Report of two cases and review of the literature. Cancer 1984;54:780-4. 5. Sanchez JA, Newman KD, Eichelberger MR, Nauta RJ. The papillary-cystic neoplasm of the pancreas. An increasingly recognized clinicopathologic entity. Arch Surg 1990;125: 1502-5.