Lipomatous Pseudohypertrophy of the Pancreas

Altinel, Deniz; Basturk, Olca; Sarmiento, Juan M.; Martín, Diego R.; Jacobs, Michael J.; Kooby, David A.; Adsay, Volkan

doi:10.1097/mpa.0b013e3181bd2923

Cited by 42 publications

(36 citation statements)

References 27 publications

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“…In fact, it is the most common degenerative lesion of the pancreas and regarded as a normal finding in the obese and elderly. 19,20 However, it typically presents focally and is not as extensive as seen in PRSS1 patients. Other conditions associated with fatty replacement and pancreatic atrophy include Cushing syndrome, steroid therapy, malnutrition, viral infections, and main pancreatic duct obstruction.…”

Section: Discussionmentioning

confidence: 99%

“…21–23 Genetic syndromes, such as Shwachman-Diamond, Bannayan, Johanson-Blizzard, and cystic fibrosis, are also associated with lipomatous atrophy. 20,24–28 It is noteworthy that the most common etiology of pancreatitis in children is cystic fibrosis, an autosomal recessive disorder caused by CFTR . In the vast majority of patients, exocrine pancreatic insufficiency occurs in utero or soon after birth, whereas 1% to 2% of patients harboring minor CFTR mutations present with idiopathic chronic pancreatitis in which the pulmonary, intestinal, and cutaneous manifestations of the disease are silent.…”

Section: Discussionmentioning

confidence: 99%

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The Histopathology of PRSS1 Hereditary Pancreatitis

Singhi

Pai

Kant

et al. 2014

American Journal of Surgical Pathology

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Hereditary pancreatitis is an autosomal dominant disorder with 80% penetrance and variable expressivity. The vast majority of cases have been linked to mutations within the cationic trypsinogen gene, also referred to as serine protease 1 (PRSS1). Other than inheritance, PRSS1 pancreatitis has been considered clinically and pathologically indistinguishable from other etiologies of chronic pancreatitis. However, to date, the histologic findings of PRSS1 pancreatitis have not been well described. We, therefore, collected pancreatic specimens from 10 PRSS1 patients of various ages and examined their clinicopathologic features. Patients at the time of resection ranged in age from 9 to 66 years (median, 29 y), with a slight female predominance (60%). All patients reported a history of intermittent abdominal pain, with an age of onset ranging from infancy to 21 years of age. Examination of the gross and microscopic findings suggested a sequential pattern of changes with increasing patient age. In pediatric patients (n=4), although in most cases the pancreas was grossly normal, there was microscopic variation in lobular size and shape. Although the central portions of the pancreas displayed parenchymal loss accompanied by loose perilobular and interlobular fibrosis, the periphery was remarkable for replacement by mature adipose tissue. These changes were more developed in younger adults (n=2), in whom fatty replacement seemed to extend from the periphery to the central portions of the pancreas. With older patients (n=4), the pancreas showed marked atrophy and extensive replacement by mature adipose tissue with scattered islets of Langerhans and rare acinar epithelium concentrated near the main pancreatic duct. In summary, PRSS1 hereditary pancreatitis is characterized by progressive lipomatous atrophy of the pancreas.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

The Histopathology of PRSS1 Hereditary Pancreatitis

Singhi

Pai

Kant

et al. 2014

American Journal of Surgical Pathology

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“…40 For lipomatous pseudohypertrophy, documentation of the demarcation of the adipose tissue from the surrounding tissue is an important clue to the diagnosis. 41 …”

Section: Dissection and Samplingmentioning

confidence: 99%

Whipple Made Simple For Surgical Pathologists

Adsay¹,

Basturk

Saka³

et al. 2014

American Journal of Surgical Pathology

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Pancreaticoduodenectomy (PD) specimens present a challenge for surgical pathologists because of the relative rarity of these specimens, combined with the anatomic complexity. Here, we describe our experience on the orientation, dissection, and sampling of PD specimens for a more practical and accurate evaluation of pancreatic, distal common bile duct (CBD), and ampullary tumors. For orientation of PDs, identification of the “trapezoid,” created by the vascular bed at the center, the pancreatic neck margin on the left, and the uncinate margin on the right, is of outmost importance in finding all the pertinent margins of the specimen including the CBD, which is located at the upper right edge of this trapezoid. After orientation, all the margins can be sampled. We submit the uncinate margin entirely as a perpendicular inked margin because this adipose tissue–rich area often reveals subtle satellite carcinomas that are grossly invisible, and, with this approach, the number of R1 resections has doubled in our experience. Then, to ensure proper identification of all lymph nodes (LNs), we utilize the orange-peeling approach, in which the soft tissue surrounding the pancreatic head is shaved off in 7 arbitrarily defined regions, which also serve as shaved samples of the so-called “peripancreatic soft tissue” that defines pT3 in the current American Joint Committee on Cancer TNM. With this approach, our LN count increased from 6 to 14 and LN positivity rate from 50% to 73%. In addition, in 90% of pancreatic ductal adenocarcinomas there are grossly undetected microfoci of carcinoma. For determination of the primary site and the extent of the tumor, we believe bisectioning of the pancreatic head, instead of axial (transverse) slicing, is the most revealing approach. In addition, documentation of the findings in the duodenal surface of the ampulla is crucial for ampullary carcinomas and their recent site-specific categorization into 4 categories. Therefore, we probe both the CBD and the pancreatic duct from distal to the ampulla and cut the pancreatic head to the ampulla at a plane that goes through both ducts. Then, we sample the bisected pancreatic head depending on the findings of the case. For example, for proper staging of ampullary carcinomas, it is imperative to take the sections perpendicular to the duodenal serosa at the “groove” area, as ampullary carcinomas often extend to this region. Amputative (axial) sectioning of the ampulla, although good for documentation of the peri-Oddi spread of the intra-ampullary tumors, unfortunately disallows documentation of mucosal spread of the papilla of Vater tumors (those arising from the edge of the ampulla, where the ducts transition to duodenal mucosa and extending) into the neighboring duodenum. Axial sectioning also often fails to document tumor spread to the “groove” area. In conclusion, knowledge of the gross characteristics of the anatomic hallmarks is essential for proper dissection of PD specimens. The approach described above allows practical and accurate documenta...

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“…In contrast, lipomatous pseudohypertrophy is a distinct entity characterized by pseudotumor formation by adipose tissue, replacing almost an entire segment of exocrine parenchyma 150 .…”

Section: Lipomatous Pseudohypertrophymentioning

confidence: 99%

Benign Tumors and Tumorlike Lesions of the Pancreas

Basturk

Aşkan

2016

Surgical Pathology Clinics

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Synopsis The pancreas is a complex organ that may give rise to large number of neoplasms and non-neoplastic lesions. This article will focus on benign neoplasms such as serous neoplasms as well as tumor-like (pseudotumoral) lesions that may be mistaken for neoplasm not only by clinicians and radiologists, but also by pathologists. The family of pancreatic pseudotumors, by a loosely defined conception of that term, includes a variety of lesions including heterotopia, hamartoma, and lipomatous pseudohypertrophy. Autoimmue pancreatitis (covered in chronic pancreatitis chapter) and paraduodenal (“groove”) pancreatitis may also lead to pseudotumor formation. Knowledge of these entities will help in making an accurate diagnosis.

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Lipomatous Pseudohypertrophy of the Pancreas

Cited by 42 publications

References 27 publications

The Histopathology of PRSS1 Hereditary Pancreatitis

The Histopathology of PRSS1 Hereditary Pancreatitis

Whipple Made Simple For Surgical Pathologists

Benign Tumors and Tumorlike Lesions of the Pancreas

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