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30 Drug Induced Mesenteric and Retroperitoneal Diseases 295
with diuretics and sulfonamides; and accumulation of toxic metabolites such as
valproic acid, didanosine, pentamidine, and tetracycline data has shown that the use
of prophylactic antibiotics plays a role in the management of acute pancreatitis.
Local complications that can arise from acute pancreatitis include necrosis,
pancreatic pseudocysts, and fluid collection. In patients who develop necrotizing
acute pancreatitis or pseudocysts, complications such as venous thrombosis,
although rare, can occur due to extrinsic compression or secondary inflammation.
This leads to mesenteric ischemia. Mesenteric edema and inflammation may occur
in the setting of acute pancreatitis as well.
Suggested Reading
1. Boley SJ, Brandt LJ, Sammartano RJ. History of mesenteric ischemia. The evolution of a
diagnosis and management. Surg Clin North Am. 1997;77:275.
2. Clair DG, Beach JM. Mesenteric Ischemia. N Engl J Med. 2016;374:959.
3. Acosta S. Epidemiology of mesenteric vascular disease: clinical implications. Semin Vasc
Surg. 2010;23:4.
4. Bobadilla JL. Mesenteric ischemia. Surg Clin North Am. 2013;93:925.
5. Anderson JE, Brown IE, Olson KA, Iverson K, Cocanour C, Galante JM. Nonocclusive
mesenteric ischemia in patients with methamphetamine use. 2017 WTA Podium Paper;
Trauma Acute Care Surg. 2017; 84(6).
6. Ofer A, Abadi S, Nitecki S, et al. Multidetector CT angiography in the evaluation of acute
mesenteric ischemia. Eur Radiol. 2009;19:24.
7. Mazzei MA, Mazzei FG, Marrelli D, et al. Computed tomographic evaluation of mesentery:
diagnostic value in acute mesenteric ischemia. J Comput Assist Tomogr. 2012;36:1.
8. Banerji A, Clark S, Blanda M, et al. Multicenter study of patients with angiotensin-converting
enzyme inhibitor-induced angioedema who present to the emergency department. Ann
Allergy Asthma Immunol. 2008;100:327.
9. Gibbs CR, Lip GY, Beevers DG. Angioedema due to ACE inhibitors: increased risk in
patients of African origin. Br J Clin Pharmacol. 1999;48:861.
10. Hoover T, Lippmann M, Grouzmann E, et al. Angiotensin converting enzyme inhibitor
induced angio-oedema: a review of the pathophysiology and risk factors. Clin Exp Allergy.
2010;40:50.
11. Tenner S, Baillie J, DeWitt J, et al. American College of Gastroenterology guideline:
management of acute pancreatitis. Am J Gastroenterol. 2013;108:1400.
12. Tadataka Y, Larson SD, Mark Evers B. Diseases of the peritoneum, retroperitoneum,
mesentery, and omentum. In: Textbook of gastroenterology, vol. 2, 5th ed. 2009. p. 2514–6.
13. Geoghegan T, Byrne AT, Benfayed W, et al. Imaging and intervention of retroperitoneal
fibrosis. Australas Radiol. 2007;51:26.
14. Alberti C. Drug-induced retroperitoneal fibrosis: short aetiopathogenetic note, from the past
times of ergot-derivatives large use to currently applied bio-pharmacology. G Chir. 2015;36
(4):187–91.
15. Elramah M, Einstein M, Mori N, Vakikl N. High mortality of cocaine-related ischemic colitis:
a hybrid cohort/case-control study. Gastrointest Endosc. 2012;75(6):1226–32.
Part VI
Neoplasms of the Mesentery
Primary Solid Neoplasms
Jasper B. van Praag, Robert C. Keskey, Eli D. Ehrenpreis,
and John C. Alverdy
Introduction
Primary tumors arising in the mesentery are relatively rare and are most often
detected incidentally by computerized tomography (CT). Because the mobility of the
mesentery permits these tumors to occupy a large anatomic space, mesenteric tumors
tend to grow to substantially large sizes. The most common symptoms and signs of
mesenteric tumors are nonspecific and include abdominal pain, weight loss, diarrhea,
and a palpable abdominal mass. Most primary neoplasms of the mesentery are
mesenchymal in origin and, in the majority of cases, are histologically benign. There
is, however, a wide variety of different types of mesenteric masses. This is due to the
many different cellular structures that make up the composition of the mesentery.
31
J. B. van Praag
Department of Surgery, University Medical Center Groningen, Groningen, The Netherlands
R. C. Keskey
Department of Surgery, University of Chicago, Chicago, IL, USA
E. D. Ehrenpreis
Department of Medicine, Advocate Lutheran General Hospital, Park Ridge, IL, USA
J. C. Alverdy (&)
Department of Surgery, University of Chicago, 5841 S. Maryland, Chicago, IL 60637, USA
e-mail: jalverdy@surgery.bsd.uchicago.edu
© Springer Nature Switzerland AG 2021
E. D. Ehrenpreis et al. (eds.), The Mesenteric Organ in Health and Disease,
https://doi.org/10.1007/978-3-030-71963-0_31
299
300 J. B. van Praag et al.
Desmoid Tumor of the Mesentery
Symptoms and Signs
Symptoms and signs of desmoid tumors are abdominal pain, a palpable abdominal
mass or complications of the mass such as gastrointestinal b leeding, small bowel
obstruction, fistula formation, or bowel perforation.
Pathophysiology
Desmoid tumors are uncommon and make up approximately 3% of all soft tissue
tumors. Mesenteric desmoid tumors are locally aggressive proliferations of
fibroblasts and can be infiltrative, but do not metastasize. They are often large in in
size when discovered and commonly obstruct or damage local bowel loops, vascular structures and/or the urinary tract. Their etiology is unknown, although
mesenteric desmoid tumors are often associated with Familiary Adenomateous
Polyposis (FAP) (Figs. 31.1 and 31.2).
Diagnosis
CT shows tend to show a well-circumscribed soft tissue mass with variable
attenuation and enhancem ent, it can demonstrate heterogeneous low attenuation
areas due to necrosis (Fig. 31.1).
Immunohistochemistry is needed for differentiation from other neoplasms.
Additionally, histologic diagnosis confirms the diagnosis of a desmoid tumor after
biopsy or removal. Histologically, desmoids are characterized by monoclonal
spindle-shaped proliferations in fibrous stroma (Fig. 31.2).
Treatment
The management of patients with mesenteric desmoid tumors is controversial as
current therapies have varying success. Surgical resection can be curative but is also
associated with significant morbidity and is associated with a high recurrence rate.
Therefore, surgery should be reserved for patients with intestinal obstruction or for
desmoid tumors that are symptomatic. These issues notwithstanding, complete
excision is often difficult. Adjuvant aggressive medical therapy with non-steroidal
anti-inflammatory agents (NSAIDs, e.g. sulindac), hormonal (tamoxifen) or cytotoxic chemotherapy (anthracyclines in combination with methotrexate and vinblastine) are most often used in lieu of surgery. Given the varying degree of
response rates and high level of recurrence, watchful waiting has been employed for
the initial management of desmoid tumors. Watchful waiting has been shown to
31 Primary Solid Neoplasms 301
Fig. 31.1 Contrast-enhanced axial CT images from a single patient with previously undiagnosed
Gardener syndrome. a shows show a well-circumscribed soft tissue mass with variable attenuation
and enhancement, it can demonstrate heterogeneous low attenuation areas due to necrosis (arrows).
b shows soft tissue attenuation in a stellate pattern within the mesenteric fat that is intimately
associated with small bowel loops (arrow). c shows innumerable polyps throughout the imaged
colon (arrows). d Most desmoid tumors will appear as well-circumscribed enhancing masses in the
mesentery or anterior abdominal wall, as it is a benign, non-inflammatory fibroblastic tumor
(asterix)
Fig. 31.2 Histology showing
fibromatosis—desmoid type
https://www.
pathologyoutlines.com/topic/
softtissuefibromatosisdeep.
html Accessed December 3rd,
2019. © Copyright
PathologyOutlines.com, Inc
302 J. B. van Praag et al.
have a similar 5-year progression free survival as those treated with medical therapy
(49.9 vs. 58.6%). Given these findings, the European Organization for Research and
Treatment of Cancer and the Soft Tissue and Bone Sarcoma Group recommend
watchful waiting for 1–2 years with frequent MRIs to elucidate the biology of the
tumor.
Prognosis
Mesenteric desmoids are potentially life threatening. Because desmoid tumors are
rare, little is known about prognosis or management options for individual cases.
They have a fairly high recurrence rate of between 25 and 60% at 5 years following
treatment. Progressive disease tends to occur when tumor size is greater than 7 cm,
has an extra-abdominal location, and in patient that is more than 37 years old.
Primary Leiomyosarcoma of the Mesentery
Symptoms and Signs
Mesenteric leiomyosarcomas are mainly asymptomatic; however, when symptoms
and signs are present, they include abdominal distension, pain and a palpable
abdominal mass. When tumors infiltrate the gastrointestinal tract, they can cause
bleeding, obstruction and pain.
Pathophysiology
Leiomyosarcoma is a mesenchymal tumor arising from smooth muscle.
Leiomyosarcoma represents 5–10% of all newly diagnosed soft tissue sarcomas.
Most often these tumors arise from the smooth muscle within the uterus, gastrointestinal tract, or blood vessels. It is believed that mesenteric leiomyosarcomas
are derived from the smooth muscle of blood vessels within the mesentery. They
have a reported incidence of only 1:350,000, most commonly occurring in
middle-aged individuals. Infrequently these tumors produce neuron-specific
enolase.
Diagnosis
Due to its frequent asymptomatic character, these neoplasms are often detected after
metastases have developed. A solid mass can be found on ultrasonography and CT,
but findings are non-specific (Fig. 31.3 ). Diagnosis can only be confirmed by
histopathology and immunological staining. Pathologically, leiomyosarcoma
31 Primary Solid Neoplasms 303
Fig. 31.3 CT scna showing a leiomyosarcoma of the mesentery located in the left upper quadrant.
Source Varghese M et al. Metastatic mesenteric dedifferentiated leiomyosarcoma: a case report and
a review of literature. Clin Sarcoma Res. 2016;6:2. Copyright © Varghese et al. 2016. Open
Access This article is distributed under the terms of the Creative Commons Attribution 4.0
International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted
use, distribution, and reproduction in any medium, provided you give appropriate credit to the
original author(s) and the source, provide a link to the Creative Commons license, and indicate if
changes were made. The Creative Commons Public Domain Dedication waiver (http://
creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article,
unless otherwise stated
Fig. 31.4 Histology showing
a leiomyosarcoma. https://
www.pathologyoutlines.com/
topic/
softtissuefibromatosisdeep.
html. Accessed December
3rd, 2019. © Copyright
PathologyOutlines.com, Inc
exhibits high mitotic activity, usually atypical. Immunohistochemistry is positive
for a-smooth muscle actin, vimentin and desmin; and stains negative for CD117
and CD34 (Fig. 31.4).
304 J. B. van Praag et al.
Treatment
Earlier detection using ultrasonography and CT, as well as complete surgical
resection, may improve long-term prognosis in patients with primary leiomyosarcoma of mesenteric origin. Surgical excision with a wide margin of normal tissue is
most effective since adjuvant chemotherapy or radiotherapy have poor responses.
Prognosis
Patients with leiomyosarcoma of the mesentery generally have a poor prognosis,
also due to late diagnosis. The overall 5-year survival rate for this tumor is only 20–
30%, and complete primary surgical resection is critical for achieving the best
outcome. Conversely, recurrence can occur within 5 years, so that close and
long-term follow-up of such patients for 5 years or more, with particular attention
to the gastrointestinal tract, liver, and lung as sites of metastasis, is required.
Prognostic factors associated with leiomyosarcomas include size >5 cm, age
>62 years, high grade lesions and incomplete excision.
Primary Carcinoid Tumors of the Mesentery
Symptoms and Signs
Cases of primary mesenteric carcinoid tumors are extremel y rare and symptoms
before diagnosis are palpable abdominal mass and non-specific abdominal pain.
These tumors can exhibit the so-called carcinoid syndrome, diarrhea and flushing
are the most prominent symptoms.
Pathophysiology
Carcinoid is used to refer to well-differentiated neuro-endocrine tumors of the
digestive tract. Mesenteric carcinoid tumors are slow-growing neoplasms that display neuroendocrine properties. They have varying degrees of fibrosis, calcification,
focal or diffuse neurovascular bundle invasion, and necrosis or lymph node
metastasis. In addition, liver metastases have been reported.
Diagnosis
Usually diagnosed by CT, mesenteric carcinoid tumors appear as a smooth-contoured
soft tissue mass surrounded by radial bands (Fig. 31.5). Primary mesenteric tumors are
diagnosed by exclusion of other conditions. Mesenteric carcinoids often (70%) have
31 Primary Solid Neoplasms 305
Fig. 31.5 A mesenteric soft tissue mass (arrows) with linear bands radiating in the surrounding
mesenteric fat is consistent with neuroendocrine tumor shown on contrast-enhanced axial and
coronal CT images
calcifications, in varying degrees. Metastatic Immunohistochemistry can show that the
tumor demonstrates positive staining for neuroendocrine markers. Biochemical
markers serum/urine 5-hydroxy indoleacteic acid (5-HIAA) and serum chromogranin
A are elevated in functioning carcinoid tumors and can be used for functional imaging
with somatostatin scan (Fig. 31.6a, b).
Treatment
No specific treatments for primary mesenteric carcinoid tumors are known. In
general, surgery of carcinoid tumors and their metastases is considered the only
curative treatment. Debulking might give relieve of symptoms due to secreted
endocrines or (intestinal) obstruction. Long-acting somatostatin analogues can also
be useful in the relief of symptoms. Other forms of treatment have not been proven
successful for these neoplasms.
Prognosis
Due to the infrequency of mesenteric carcinoid tumors, prediction of prognosis is
difficult. However, surgical resection of the tumor and/or its metastases are known
to prolong survival.
306 J. B. van Praag et al.
Fig. 31.6 Coronal fused CT (a)/PET (b) image demonstrates a small markedly avid lesion in the
pelvis just to the right of midline (arrow). Maximum intensity projection (MIP) image
demonstrates this and two other smaller foci located more inferiorly(arrows). The radiopharmaceutical (Dotatate) is a somatostatin receptor antagonist, and it is therefore highly sensitive for
detection of neuroendocrine tumors (i.e., carcinoid). This agent is eliminated really and
physiologic activity is present in the kidneys, collecting systems, and urinary bladder on the
coronal maximum-intensity projection (MIP) image
Fig. 31.7 a Coronal contrast-enhanced computed tomography and axial T2-weighted magnetic
resonance imaging demonstrate an ill-de fined, heterogeneously enhancing GIST (G) with a large
cystic component (asterisk). Additional heterogeneous soft tissue along the periphery of the lesion
is suggestive of peritoneal sarcomatosis and associated ascites (b). Layering T2-hypointense signal
(arrows) is indicative of necrosis with layering blood products. With permission from Dr. Abraham
Dachman, Dr. Scott Sorensen and Dr. Justin Ramirez