Pancreatology 13 (2013) 631e633

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Case report

Pancreatic metastasis from a solitary fibrous tumor of the kidney: A rare cause of acute recurrent pancreatitis Yuval A. Patel a, *, Sameer Dhalla b, Matthew T. Olson c, Anne Marie Lennon b, Mouen A. Khashab b, Vikesh K. Singh b a b c

Department of Medicine, Johns Hopkins Medical Institutions, Baltimore, MD, USA Division of Gastroenterology, Department of Medicine, Johns Hopkins Medical Institutions, Baltimore, MD, USA Department of Pathology, Johns Hopkins Medical Institutions, MD, USA

a r t i c l e i n f o

a b s t r a c t

Article history: Received 4 May 2013 Received in revised form 12 June 2013 Accepted 13 June 2013

Solitary fibrous tumors are unusual spindle cell neoplasms that uncommonly originate from the kidney. We report a case of a 43-year old male who presented with acute recurrent pancreatitis secondary to a mass in the head of the pancreas. Endoscopic ultrasound with fine needle aspiration (EUS-FNA) was performed. Cytology revealed solitary fibrous tumor of the kidney. This is the first reported case of solitary fibrous tumor metastasizing to the pancreas and presenting as acute recurrent pancreatitis. Copyright Ó 2013, IAP and EPC. Published by Elsevier India, a division of Reed Elsevier India Pvt. Ltd. All rights reserved.

Keywords: Recurrent pancreatitis Pancreatic metastases Solitary fibrous tumor

1. Introduction Solitary fibrous tumors (formerly known as hemangiopericytomas) are uncommon spindle cell neoplasms that typically occur in the pleura but can be observed in extrapleural sites, including soft tissue and visceral organs [1]. Urogenital occurrence is rare with only 38 cases of solitary fibrous tumor of the kidney reported in the literature [2]. Solitary fibrous tumors are generally slow-growing tumors with a favorable prognosis. Approximately 10e15% of these neoplasms are malignant [3]. While the prognosis of solitary fibrous tumors of the kidney is unknown, the 5-year disease-free survival of solitary fibrous tumors of the pleura is 73% for benign and 46% for malignant lesions [4]. We report a case of solitary fibrous tumor of the kidney which metastasized to the pancreas and resulted in acute recurrent pancreatitis. This was diagnosed using endoscopic ultrasound with fine-needle aspiration (EUS-FNA). 2. Case report A 43-year-old-male presented for evaluation of acute recurrent pancreatitis. He denied any history of smoking and reported only

* Corresponding author. E-mail address: [email protected] (Y.A. Patel).

rare alcohol use. He had a history of biliary colic for which he underwent a laparoscopic cholecystectomy in 2002. On abdominal imaging, he was found to have an incidental left kidney mass and underwent a left nephrectomy in 2002. The surgical histopathology demonstrated a benign solitary fibrous tumor. He was in his usual state of health until 2011 when he had 4 episodes of unexplained acute pancreatitis over a 6 month period. He did not seek medical attention until the third episode. An abdominal CT scan revealed inflammation in the fat surrounding the pancreas with a heterogeneous collection in the head of the pancreas initially concerning for a pseudocyst with hemorrhage or a pseudoaneurysm. During his fourth episode of acute pancreatitis, an MRI/MRCP was obtained and revealed a 5.8  6.0 cm heterogeneous mass in the head of the pancreas which abutted the inferior vena cava and portal confluence (Fig. 1). There was a 3  2.7 cm liver lesion suspicious for a solitary metastasis. The bile duct was displaced laterally but not obstructed. The superior mesenteric and celiac arteries were patent. The patient underwent EUS-FNA which demonstrated a 5.7  5.2 cm well-circumscribed heterogeneous mass with numerous anechoic areas consistent with cysts (Fig. 2). On doppler ultrasound there was evidence of multiple vessels associated with the mass. The remaining parenchyma in the body and tail demonstrated focal lobularity and diffuse echogenic foci and >2 mm strands. The pancreatic duct was not dilated. There were no parenchymal calcifications or lymphadenopathy. The solitary liver lesion on MRI was not appreciated on EUS.

1424-3903/$ e see front matter Copyright Ó 2013, IAP and EPC. Published by Elsevier India, a division of Reed Elsevier India Pvt. Ltd. All rights reserved. http://dx.doi.org/10.1016/j.pan.2013.06.004

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Y.A. Patel et al. / Pancreatology 13 (2013) 631e633

Fig. 1. MRI/MRCP: Green arrow e displaced bile duct; Yellow arrow e pancreatic duct; Red arrow e pancreatic mass.

Cytology revealed a sparse population of spindle and epithelioid cells, some of which were associated with large vessels (Fig. 3). The cell block demonstrated staghorn vessel appearance (Fig. 4) which is characteristic of solitary fibrous tumors. Immunohistochemical staining demonstrated strong and diffuse staining for CD99 and CD34 as well as focal immunoreactivity for Bcl-2, which is also consistent with solitary fibrous tumor. The patient underwent a classic pancreaticoduodenectomy (Whipple) and partial hepatectomy for a single liver metastasis with curative intent. The surgical histopathology confirmed a solitary fibrous tumor. He did well after surgery, only suffering a minor pancreaticojejunostomy leak that resolved after drain placement. He has been followed with serial CT and MRI studies which show no recurrence of disease after one and a half years. He is clinically well and has had no further episodes of acute pancreatitis. 3. Discussion Solitary fibrous tumors of the kidney are very rare, with only 38 cases reported in the literature [2]. To our knowledge, this is the first report of solitary fibrous tumor of the kidney metastasizing to the pancreas. There is a wide differential diagnosis for pancreatic cystic masses including intraductal papillary mucinous neoplasms, solid pseudopapillary tumors, mucinous cystadenoma, neuroendocrine tumors, and cystic degeneration of a solid tumor such as mucinous cystadenocarcinoma [5]. Morphological features of pancreatic cysts that are associated with malignancy include thickened cyst walls, large size (>3 cm), dilated pancreatic duct, non-enhancing mural nodules, abrupt change in pancreatic duct caliber with distal pancreatic atrophy, and lymphadenopathy [5].

Fig. 2. EUS.

Fig. 3. Direct smears of the FNA material demonstrated spindle (red arrows) and epithelioid cells (green arrows) with conspicuous vessels (blue arrows). DiffQuik 400.

The characteristics in our case that were suspicious for malignancy included size, the presence of solid components, and thickened walls. Obstructing pancreatobiliary tumors are an uncommon cause of acute recurrent pancreatitis [6]. These tumors are most often considered in patients who are 40 years and older and present with acute pancreatitis [7]. Factors that raise concern for an underlying malignancy include new-onset diabetes, weight loss, and history of tobacco use [8]. The most common tumors causing acute recurrent pancreatitis are intraductal papillary mucinous neoplasms, ampullary tumors, pancreatic adenocarcinoma, and islet cell tumors [7]. Common metastatic tumors include malignant melanoma, hypernephroma, and lymphoma [9]. It is often difficult to appreciate an underlying tumor during the period of active or resolving acute pancreatitis. This occurred in our patient as the initial CT scan of the abdomen was concerning for a pseudocyst or pseudoaneurysm as opposed to a cystic mass. Given the possibility of missing a lesion, additional abdominal imaging and close followup should be pursued, especially in those patients with risk factors for malignancy. In our case, MRI/MRCP was subsequently performed and better characterized the pancreatic lesion as a cystic mass. This was followed by EUS-FNA, as this modality has been shown to incrementally increase the overall accuracy for diagnosis

Fig. 4. Cell-block preparation of the FNA material demonstrated staghorn vessels. H&E, 400.

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of neoplastic pancreatic cysts [10]. EUS-FNA was important in our patient as pre-operative diagnosis of solitary fibrous tumor with a known liver metastasis would make surgery with curative intent a viable option. The cytology in our case demonstrated strong and diffuse staining for CD99 and CD34, focal immunoreactivity for Bcl-2, and negative staining for CD117 (C-KIT), which was consistent with solitary fibrous tumor. Chromogranin and synaptophysin stains, often seen in neuroendocrine tumors, were also negative. Complete surgical resection with clear surgical margins is the gold-standard for cure in patients with solitary fibrous tumors [11]. A poorer prognosis is related to incomplete resection. There are case series which support the role of adjuvant chemotherapy or radiation for locally-advanced or metastatic tumors. However, there is no widely accepted approach to the management of these patients after surgical resection [12]. Recurrence occurs in about 10e15% of patients after complete resection [2]. These tumors are pathologically classified as typical or malignant based on mitotic count, evidence of necrosis, and nuclear polymorphism [13]. A strong correlation between pathology and clinical course does not appear to exist as pathologic evolution may occur in patients with recurrence from an originally typical tumor [13]. The histopathology in the present case demonstrated evolution toward malignant behavior with increased mitotic index as compared to the benign histopathology noted on nephrectomy 9 years earlier. Late recurrence (10 years) constitutes about 10% of total recurrences as noted in one case series, appearing to result from this evolution toward malignant pathology [13]. The risk for recurrence for our patient given his additional solitary liver lesion is unknown. There are only a few case reports of solitary fibrous tumor describing local recurrence after resection of a solitary liver lesion and even fewer describing metastases [12]. Given the 9-year interval between his nephrectomy and presentation with metastatic recurrence to his pancreas and liver, the patient is being followed closely by oncology and monitored routinely with serial abdominal imaging.

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Acknowledgments No funding was required. No conflicts of interest.

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Pancreatic metastasis from a solitary fibrous tumor of the kidney: a rare cause of acute recurrent pancreatitis.

Solitary fibrous tumors are unusual spindle cell neoplasms that uncommonly originate from the kidney. We report a case of a 43-year old male who prese...
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