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31 Primary Solid Neoplasms 307
Primary Liposarcoma of the Mesentery
Symptoms and Signs
Patients may present with complaints of gradual abdominal distention, palpable
abdominal mass, abdominal pain and weight loss.
Pathophysiology
As discussed above, soft tissue neoplasms are classified based on their tissue of
origin. The mesenteric variant is rare, but liposarcoma is a common mesenchymal
malignancy usually occurring within the retroperitoneum. These tumors are locally
aggressive, are composed of lipoblasts and can secrete substances such as
granulocyte-colony-stimulating factor (G-CSF) There are several histologic subtypes that vary by histologic grade. The most common variant is the
well-differentiated (low-grade) liposarcoma. The second most common variant is
dedifferentiated liposarcoma. This tumor contains regions of non-lipogenic sarcomatous tissue within a well-differentiated tumor. The dedifferentiated subtype of
liposarcoma can be associated with distant metastasis. Liposarcomas are usually
avascular to moderately vascular and can cause displacement of the major vessels.
Diagnosis
CT images show inhomogeneity, infiltration or poor margination and contrast
enhancement. The density of the lesion exceeds that of subcutaneous fat. The
enhancement on CT changes vary with the degree of histological grade.
Well-differentiated liposarcomas are hyperintense on T2-weighted MRI with minimal or no enhancement.
Treatment
Surgical resection with sufficient surgical margin is the treatment of choice.
Although the role of additional treatment is not clear , adjuvant therapy with
chemotherapy consisting of doxorubicin, cisplatin and ifosfamide might cause
shrinkage of tumor size.
Prognosis
The prognosis of mesenteric liposarcomas is unclear but appears to depend on the
degree of cellular differentiation. In general, prognostication and stage are based on
308 J. B. van Praag et al.
both tumor size and its histologic grade. Obtaining clear margins during resection is
essential as these tumors have a high risk of local recurrence even after apparently
complete resection.
Primary Stromal Tumor of the Mesentery
Symptoms and Signs
Symptoms are generally non-specific. Signs of extra-gastrointestinal stromal tumors
(EGIST) of the mesentery include abdominal distension that gradually increases
with tumor development.
Pathophysiology
Gastrointestinal stromal (GIST) tumours are mesenchymal tumors that arise from
stromal cells, particularly interstitial cells of Cajal. EGIST of the mesentery are very
rare. EGIST generally originate from the omentum, peritoneum, and mesentery.
Based on histology and immunophenotyped stromal tumors of the mesentery are
identical to gastrointestinal stromal tumors. The tumors are CD117 (C Kit) and
CD34 positive mesenchymal neoplasms and are suggested to deriv e from
pluripotential precursor cells.
Diagnosis
On CT scan, mesenteric GIST is a well-defined lobular mass showing heterogeneous contrast enhancement with areas of hypodensity (See Fig. 31.7a, b). On
histological examination, spindle cells with eosinophilic cytoplasm are observed
and are CD117 positive, CD34 positive, vimentin positive, S100 negative, and
desmin negative (Fig. 31.8).
Treatment
The treatment of mesenteric stromal tumors is the perfor mance of an en bloc
resection, especially when tumors are >2 cm in size. Given the high level of KIT
mutations, tyrosine kinase inhibitor drug treatment has been proven to prolong
survival. Imatanib can be given in both the adjuvant and neoadjuvant setting. In the
adjuvant setting, trials have shown a significant disease-free survival for GISTs
<2 cm. Adjuvant treatment has been shown to improve disease free survival and
should be administered for three years post resection in high risk GISTs. Whether
treatment should be continued longer than 3 years has not been determined. In the
31 Primary Solid Neoplasms 309
Fig. 31.8 H&E staining of a GIST tumor demonstrating uniform spindle cells with elongated
nuclei and eosinophilic cytoplasm. Reused with permission Reproduced with permission from:
Morgan J, Raut CP, Duensing A, Keedy VL. Epidemiology, classification, clinical presentation,
prognostic features, and diagnostic work-up of gastrointestinal stromal tumors (GIST). In:
UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on [Date].) Copyright © 2020
UpToDate, Inc. For more information visit www.uptodate.com
neoadjuvant setting, some studies have shown imatinib can be used to decrease
tumor size. This in turn can improve the ability to obtain negative margins and
decrease the morbidity of the surgery. In the setting of metastatic GISTs, ima tinib
has become first line treatment. There is some question whether or not resection has
some benefit in the setting of metastatic GISTs.
Prognosis
For GIST tumors, prognosis is largely determined by location, size of the tumor,
and the mitotic rate (uniquely measured in GISTs). GISTs tend to have a high
recurrence rate, and EGIST arising from the mesentery tend to have less favorable
outcomes. Predictive models exist that are largely based on tumor size and mitotic
rate. These prediction models can be used to predict the need for adjuvant therapy
for imatinib. There is limited data on the prognosis of EGIST, given its low
prevalence. However, models may also be used to estimate risk. These include the
general understanding that mesenteric GISTs tend to have a poor prognosis.
310 J. B. van Praag et al.
Summary
Primary tumors of the mesentery are rare and often present as incidental findings on
CT scan. All tumors should be aggressively worked up with biopsy if indicated.
Prognosis for tumors are dependent on the tumor biology, extent of disease and
completeness of resection. Adjuvant chemotherapy is needed in most cases.
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2. Sheth S, Horton KM, Garland MR, Fishman EK. Mesenteric neoplasms: CT appearances of
primary and secondary tumors and differential diagnosis. RadioGraphics. Radiological
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J Visc Surg. 2012;149(4):e239–51.
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pathological processes. Eur Radiol. 1998;8(6):886–900.
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Pathol. 1996;(9):939.
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treatment. Int J Colorectal Dis. 1989;4(1):30–6.
8. Jalini L, Hemming D, Bhattacharya V. Intraabdominal desmoid tumour presenting with
perforation. Surgeon. 2006;4(2):114–6.
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tumors. Ann Surg Oncol. 2016;23(7):2212–9.
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correlation with progression-free survival. Clin Cancer Res. 2015;21(18):4194–200.
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(11):1494–504.
13. Mizoe A, Takebe K, Kanematsu T. Primary leiomyosarcoma of the jejunal mesentery: report
of a case. Surg Today. Springer; 1998;28(1):87–90.
14. Fukunaga M. Neuron-specific enolase-producing leiomyosarcoma of the mesentery. Case
report. APMIS. Wiley/Blackwell (10.1111); 2004;112(2):105–8.
15. Hashimoto H, Tsuneyoshi M, Enjoji M. Malignant smooth muscle tumors of the
retroperitoneum and mesentery: a clinicopathologic analysis of 44 cases. J Surg Oncol.
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16. Koczkowska M, Lipska BS, Grzeszewska J, Limon J, Biernat W, Jassem J. Primary
leiomyosarcoma of the mesentery in two sisters: clinical and molecular characteristics. Pol J
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and medical aspect of the midgut carcinoids. Cancer Treat Rev. 2002;28(1):11–25.
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fibrosis in mesenteric carcinoid tumor: CT findings and pathologic correlation. Am J
Roentgenol. American Public Health Association; 1995;164(2):387–91.
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UpToDate Nov 2019. Accessed 12 May 2019.
Metastatic Diseases of the Mesentery
John C. Alverdy and Robert C. Keskey
Symptoms
As with other mesenteric masses, metastasis to the mesentery are initially indolent
and may be asymptomatic while they are small. When symptoms develop from
mesenteric masses, they may include the development of abdominal pain from
compression of vascular structures or intestinal obstruction. Other symptoms and
signs of obstructive symptoms may include nausea, emesis, abdominal distension,
constipation, and obstipation. As with any malignancy, there is also the propensity
to develop constitutional symptoms including fatigue and weight loss. In special
circumstances, the presence of a neuroendocrine tumor may result in the development of carcinoid syndrome (represented by flushing and diarrhea). (See
Chap. 31, Mesenteric Neoplasms).
32
Pathophysiology
Metastasis to the mesentery occur most commonly from a small intestinal neoplasms that spread along mesenteric lymphatic channels. Mesenteric metastases are
seen in small bowel adenocarcinoma and more commonly in small bowel neuroendocrine tumors. Bulky mesenteric nodal disease can cause compression of
mesenteric vessels and result in the development of chronic intestinal ischemia or
bowel obstruction.
J. C. Alverdy (&) R. C. Keskey
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_32
313
314 J. C. Alverdy and R. C. Keskey
Metastatic invasion of the mesentery can also occur in conjun ction with
abdominal carcinomatosis. Peritoneal carcinomatosis occurs when either gastrointestinal (typically colorectal or gastric cancer), gynecological (ovarian cancer), or
primary peritoneal malignancies (mesothelioma, pseudomyxoma peritonei) disseminate throughout the peritoneal cavity. Carcinomatosis occurring from gynecological or gastrointestinal malignancies, occurs as a result of tumor penetration
through the serosa of the GI tract or cortex of the ovary. Carcinomatosis can involve
any surface throughout the peritoneal cavity including the mesentery.
Diagnosis
Metastatic disease from lymphatic spread of small bowel tumors is diagnosed in a
similar manner as other mesenteric masses using cross sectional imaging with
computerized tomography (CT), or magnetic resonance imaging (MRI) with or
without positron emission tomography (PET). Depending on the degree of lymphatic invasion, there may be some associated imaging signs of mesenteric ischemia associated with the metastatic lesions. The diagnosis of ischemia however
cannot be definitively until tissue is visualized, and resected or biopsied. Carcinomatosis can be difficult to detect on imaging and may require diagnostic
laparoscopy for definitive diagnosis. Some signs of carcinomatosis that may be
visualized on imaging studies include scalloping of the liver and spleen and the
presence of ascites. Occasionally, omental thickening can be detected as evidence
of disease localizing to the omentum (Fig. 32.1a, b).
Fig. 32.1 a CT showing peritoneal carcinomatosis. Irregular soft tissue of the omentum (*) and
nodular thickening of the mesenteric root (arrows). b CT demonstrating enhancing of soft tissue
nodules anterior to the liver and spleen (black arrows). With permission from Dr. Abraham
Dachman, Dr. Scott Sorensen and Dr. Justin Ramirez
32 Metastatic Diseases of the Mesentery 315
Treatment
For metastases to the mesenteric lymph nodes, resection of the mass along with
adjacent segment of bowel should be performed. In the setting of peritoneal carcinomatosis, cytoreductive surgery and HIPEC (intraperitoneal hypothermic
chemotherapy) have been found to provide survival benefit for some cases
depending on the extent of the disease and type of malignancy.
Malignant Bowel Obstructions and Palliative Care
When a patient is diagnosed with an intra-abdominal malignancy, goals of care
should be established with the patient and their primary decision makers. In this
setting, the main goals of treatment are to obtain symptomatic relief, especially in
the case of peritoneal carcinomatosis as the overall prognosis in these patients is
generally very poor. For mesenteric neoplasms, extra-luminal compression of the
intestine can result in bowel obstruction. Malignant bowel obstruction manifests as
nausea, vomiting, and abdominal pain. When a patient is diagnosed with malignant
bowel obstruction, the overall predicted survival is about 125 days, with 42%
patients dying within 90 days of surgical consultation.
Initial management of a malignant bowel obstruction from a mesenteric neoplasm should be nasogastric tube decompression to alleviate gastric distension from
a distal obstruction. By decreasing gastric distension, patients tend to have rapid
improvement in nausea. Placement of an endoscopic gastrostomy tube can be
considered for long-term management in these patients, especially when medical
therapy of nausea fails. Placement of a gastrostomy tube allows for periodic venting
and relief of gastric distension. Retrospective studies have demonstrated that
patients who have venting gastrostomy tubes have fewer hospital readmissions and
a lower rate of in-hospital deaths. Another consideration for palliative care in this
patient group is the performance of colonic stenting to help alleviate obstruction.
The performance of colonic stenting in individual patients is largely depend on the
anatomic location of the obstruction and the availability of skilled endoscopists.
Stenting offers a short-term solution to malignant obstructions; however, up to one
third of patients with colonic stents will develop recurrent obstruction and require
repeated intervention. Both endoscopic gastrostomy tubes and colonic stents have
significant potential complications but may provide a means for achieving end of
life goals.
The mainstay of pharmacologic management of malignant bowel obstruction is
anti-secretory therapy. Octreotide, a somatostatin analog is generally the first line
treatment for decreasing intestinal secretions. Randomized controlled trials have
316 J. C. Alverdy and R. C. Keskey
shown that somatostatin analogs are more effective at relieving symptoms in
malignant bowel obstructions than anticholinergic medications. Long acting depot
injections of somatostatin analogs can be considered for outpatient treatment. Some
anticholinergic medications that are also used include hyoscine, butylbromide, and
glycopyrrolate. These are thought to have fewer central nervous system side effects
than other anticholinergic agents. Glucocorticoids represents another adjunctive
measure for patients with mesenteric metastatic disease. However, there is limited
evidence regarding the efficacy of glucocorticoid therapy in this clinical setting.
Conclusion
Metastatic disease to the mesentery should be strongly considered in patients who
develop a mesenteric mass. Metastases to the mesentery occur from lymph node
drainage from adjacent small bowel malignancies or from peritoneal carcinomatosis. In either case, depending on the extent of disease, surgical resection has a
significant potential role in patient management. Finally, palliative options are
available for patients with malignant bowel obstruction from mesenteric metastases.
These treatments should be considered to improve their quality of life.
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Mesenteric Lymphoma
Emily Papazian, Robert C. Keskey, and John C. Alverdy
Symptoms and Signs
Mesenteric lymphoma commonly presents with abdominal pain and presence of a
palpable abdominal mass. Advanced stage disease presents with systemic symptoms including fever, night sweats and weight loss and may rarely result in complications such as intestinal obstruction, perforation and hemorrhage. Mesenteric
lymphoma may also present with hepatosplenomegaly. Thus, evaluation of the size
and consistency of the liver and spleen should be evaluated along with the presence
of peripheral lymph nodes in staging of the disease.
Pathophysiology
33
Lymphoma is the most common solid mesenteric tumor. Lymphomas are broadly
categorized into two main subtypes: Non-Hodgkin’ s lymphoma and Hodgkin’s
lymphoma. Non-Hodgkin’s lymphoma is usually of B cell origin. The disease is
often diagnosed with a peripheral lymph node biopsy and disease in the abdomen is
presumed to be due to systemic disease. Disease may be detected in mesenteric
lymph nodes in approximately 30–50% of patients with non-Hodgkin’s lymphoma,
of which 40–67% are large B cell tumors. Spread to the mesentery should especially be considered in patients with para-aortic disease.
E. Papazian R. C. Keskey 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_33
317