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484 R. Sehgal et al.
Prognosis depends on the histological subtype. In 1979, Evans reported that the
median survival for patients with the well-differentiated, myxoid, dedifferentiated
and pleomorphic types was 119, 113, 59 and 24 months, respectively.
Castleman’s Disease
Castleman’s disease is a rare benign lymphoproliferative disorders that affects
adults in the third and fourth decade of life. It was first described by the American
physician and pathologist Dr. Benjamin Castleman in 1956 when he described a
group of patients with large thymoma-like masses in the anterior mediastinum. The
aetiology is unknown, however it has been shown associated with the human
immunodeficiency virus (HIV) and human herpes virus 8 (HHV8). Moreover it has
been linked to several malignancies such as Kaposi’s sarcoma, non-Hodgkin’s
lymphoma, Hodgkin’s lymphoma and POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin manifestation). Although Castleman’s
disease usually involves the mediastinum in 70% of cases, reports of extrathoracic
lesions involving the neck, axilla, shoulders, pelvis, pancreas, nasopharynx and
retroperitoneum have been described. Castleman’s disease involving the mesentery
is a rare.
Castleman’s disease can be classified into two histological types, hyaline type
and plasma cell type. The hyaline type is typified by the presence of small hyaline
follicles and intrafollicular capillary proliferation. This type account s for more than
90% of Castleman’s disease cases, usually presents with localized disease (unicenteric) and is asymptomatic in the majority. The plasma cell type is less common
and usually presents with disseminated lymphadenopathy (i.e. multicenteric),
pyrexia, diaphoresis, weight loss, anaemia, elevated C-reactive protein (CRP),
erythrocyte sedimentation rate (ESR) and hypergammaglobulinemia. However the
hyaline and plasma cell type can exhibit considerable and histologic overlap, thus a
so-called mixed variant of Castleman’s disease is occasionally seen.
Radiological investigations are nonspecific and not diagnostic. Abdominal
ultrasound can detect homogenous, hypoechoic mass like lesions that point towards
Castleman’s disease. The classic CT appearance of hyaline Castleman’s disease is
that of a solitary enlarged lymph node or localized nodal masses demonstrating
homogeneous, inte nse contract enhancement. Three patterns of disease have been
described; these include a solitary non-invasive mass (most common: 50% of
cases), a dominant infiltrative mass with associated lymphadenopathy (40% of
cases), and matted lymphadenopathy without a dominant mass (10% of cases). On
magnetic resonance imaging (MRI), lesions in hyaline vascular Castleman’s disease
classically exhibit heterogeneous T1 and T2 hyperintensity compared with skeletal
muscle. Prominent flow voids may be seen, identifying feeding vessels. MRI
enables assessment of the extent of disease as well as its relationship to adjacent
structures (although evaluation of calcification is limited.
48 Mesenteric Neoplasms 485
Due to lack of specific radiographic features for Castleman’s disease CT or
ultrasound-guided fine-needle aspiration of the mass lesion should be performed to
obtain an accurate tissue diagnosis. Solitary lesions or unicentric type Castleman’s
disease virtually always behave in a benign manner. Indications for surgery include
the presence of focal lesions suited to curative resections, or multicentric lesions, for
palliative resection or attempt for curative resection with multimodal medical and
chemotherapy. Surgical excision of focal mesenteric lesions is recommended and
associated with excellent prognosis. Recurrence of localised disease is rare after
complete surgical resection, but has been reported in patients after incomplete
surgical removal. Limited success with radiotherapy (30–45 Gy) and chemotherapy
has been reported. Multicentric Castleman’s disease carries a poor prognos is as the
disease is systemic. Management is controversial for multicentric lesions and is
largely considered multimodal encompassing surgery, chemotherapy, ster oid therapy, antiviral medication, or the use of antiproliferative regimens. In most cases,
diagnosis of mesenteric Castle man’ s disease is confirmed only after resection and
histopathological examination of the specimen.
Cystic Lesions
Mesenteric cysts are benign intra-abdominal lesions first described in 1507 by the
Florentine anatomist Benevenni while performing an autopsy in an 8-year old boy.
This was followed by the first description of a chylous cyst by von Rotitansky in
1842. In 1852, Gairdner described an omental cyst. Tillaux described the first
successful surgical treatment of a mesenteric cyst in 1880. He was succeeded by
Pean in 1883, who described the first marsupialization technique for mesenteric
cysts. However, it was not until 1993 that Mackenzie reported the first successful
laparoscopic resection of a mesenteric cyst.
Mesenteric cysts have an incidence ranging from 1 in 100,000 in the adult
population and 1 in 20,000 in the pediatric population. They affect females more
than males the fourth decade of life. The majority of mesenteric cysts are single
however can be uni- or multi-locular. They are asymptomatic in approximately 45%
of cases and diagnosed incidentally during routine physical examination, during
abdominal surgery, or routine imaging. However they can present with a variety of
nonspecific symptoms such as abdominal pain, distension, palpable mass, nausea,
vomiting, change of bowel habit and weight loss. Up to one third of patients present
acutely due to complications from the cyst. These include intestinal obstruction,
volvulus, haemorrhage, infection or cyst rupture.
Mesenteric cysts range between 2 to 35 cm in size and the majority (50–67%) of
reported cases are in the small bowel mesentery. This is followed by 24–37%
located in the mesocolon and the rema inder within the retroperitoneum. The
majority are solitary lesions however uni- and multilocular varieties exist. Most are
comprised of a single layer of columnar or cuboidal epithelial cells. The cyst fluid
can be chylous, serous, or haemorrhagic and associated with small bowel
486 R. Sehgal et al.
mesentery, mesocolon and trauma respectively. Mesenteric cysts can be classified
based on histological features into six subgroups: (1) cysts of lymphatic origin
(simple lymphatic cyst and lymphangioma); (2) cysts of mesothelial origin (simple
mesothelial cyst, benign cystic mesothelioma and malignant cystic mesothelioma);
(3) cysts of enteric origin (enteric cyst and enteric duplication cyst); (4) cysts of
urogenital origin; (5) mature cystic teratoma (dermoidcysts); and (6) pseudocysts
(infectious and traumatic cysts).
Ultrasound and CT are the main imaging modalities utilized characterizing
mesenteric cysts. Ultrasound has a higher sensitivity in characterizing the internal
nature of the cyst and can differentiate between septations, debris, and fluid levels.
CT will provide greater detail regarding location of the cyst and its relation to
surrounding structures. Mesenteric cysts carry a 3% overall malignant potential.
Majority of cancers developing out of mesenteric cysts are sarcomas with a few
cases describing adenocarcinomas. Poor prognosticators on radiological imagining
include rapid growing cysts that possess a solid component within the cyst.
Surgery with complete enucleation is the recommended treatment of choice for
mesenteric cysts. Older techniques such as simple aspiration and marsupialization
are associated with greater recurrence and infection rates. Depending on the association of the cyst to surrounding structures, an en-bloc resection incorporating
bowel may be necessary. While performing surgery either open or via the minimally invasive approach it is recommended to abide to fundamental oncological
principles to prevent recurr ence. In the setting of managing mesenteric cysts conservatively no clear consensus exists regarding follow-up. Several authors have
recommended frequent imaging for cysts that are bigger in size, possess a solid
component within the cyst and have a faster rate of growth as such would be
indicative of increased malignant potential and the risk of developing
complications.
The prognosis for mesenteric cysts is generally good as the majority are benign
and recurrence rates are low with a R0 resection. Recurrence is greater in those
cysts located in the retroperitoneum as they are more technically demanding to
excise due to their close proximity to great vessels, ureter and other organs.
Conclusions
With the new understanding of the mesentery as a unique continuous organ, we can
review the disease states that involve the mesentery. While rare overall, the basic
understanding of the primary lesions of the mesentery, their distinguishing features,
diagnosis and management options, is key to optimize management of mesenteric
neoplasms.
48 Mesenteric Neoplasms 487
Suggested Reading
1. Coffey JC, O’Leary DP. The mesentery: structure, function, and role in disease. The Lancet
Gastroen Hepatol. 2016;1(3):238–47.
2. Coffey JC, Sehgal R, Walsh D. Pathology of the mesentery. Mesenteric Principles of
Gastrointestinal Surgery: Basic and Applied Science: CRC Press; 2017. p. 85–109.
3. Dufay C, Abdelli A, Le Pennec V, Chiche L. Mesenteric tumors: diagnosis and treatment.
J Visc Surg. 2012;149(4):e239–51.
4. Mueller PR, Ferrucci JT Jr, Harbin WP, Kirkpatrick RH, Simeone JF, Wittenberg J. Appearance of lymphomatous involvement of the mesentery by ultrasonography and body computed
tomography: the “sandwich sign” . Radiology. 1980;134(2):467–73.
5. Salemis NS, Gourgiotis S, Tsiambas E, Karagkiouzis G, Nakos G, Karathanasis V. Diffuse
large B cell lymphoma of the mesentery: an unusual presentation and review of the literature.
J Gastroint Cancer. 2009;40(3–4):79–82.
6. Kasper B, Strobel P, Hohenberger P. Desmoid tumors: clinical features and treatment options
for advanced disease. Oncologist. 2011;16(5):682–93.
7. Bonvalot S, Desai A, Coppola S, Le Péchoux C, Terrier P, Dômont J, et al. The treatment of
desmoid tumors: a stepwise clinical approach. Annals Oncol. 2012;23(suppl_10):x158−66.
8. Mazur MT, Clark HB. Gastric stromal tumors. Reappraisal of histogenesis. Am J Surg Pathol.
1983;7(6):507–19.
9. Schwameis K, Fochtmann A, Schwameis M, Asari R, Schur S, Köstler W, et al. Surgical
treatment of GIST—an institutional experience of a high-volume center. Int J Surg. 2013;11
(9):801–6.
10. Dematteo RP, Ballman KV, Antonescu CR, Maki RG, Pisters PW, Demetri GD, et al.
Adjuvant imatinib mesylate after resection of localised, primary gastrointestinal stromal
tumour: a randomised, double-blind, placebo-controlled trial. Lancet (London, England).
2009;373(9669):1097–104.
11. Modlin IM, Kidd M, Latich I, Zikusoka MN, Shapiro MD. Current status of gastrointestinal
carcinoids. Gastroenterology. 2005;128(6):1717–51.
12. Jain SK, Mitra A, Kaza RC, Malagi S. Primary mesenteric liposarcoma: an unusual
presentation of a rare condition. J Gastrointest Oncol. 2012;3(2):147–50.
13. Moyana TN. Primary mesenteric liposarcoma. Am J Gastroent. 1988;83(1):89–92.
14. Soft tissue and visceral sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment
and follow-up. Annals Oncol Official J Eur Soc Med Oncol. 2012;23 Suppl 7:vii92–9.
15. Chronowski GM, Ha CS, Wilder RB, Cabanillas F, Manning J, Cox JD. Treatment of
unicentric and multicentric Castleman disease and the role of radiotherapy. Cancer. 2001;92
(3):670–6.
16. de Perrot M, Brundler M, Totsch M, Mentha G, Morel P. Mesenteric cysts. Toward less
confusion? Dig Surg. 2000;17(4):323–8.
Mesenteric Artery Thrombosis and Embolism
Salvatore Parascandola and Vincent Obias
Description of This Condition
Mesenteric vascular occlusive disease is a rare but potentially devastating condition,
characterized by intestinal hypoperfusion of the small intestine caused by complete
or partial obstruction of the mesenteric arteries. Mesenteric ischemia accounts for
roughly 1 in every 1000 hospital admissions in this country, with mortality rates
ranging as high as 50–75%. Untreated, acute mesenteric ischemia carries a mortality of close to 100%. The most common causes of acute mesenteric ische mia
(AMI) are arterial embolism (40 to 50%) and arterial thrombosis (25 to 30%).
49
Anatomy and Pathophysiology
Perfusion to the gastrointestinal system is provided by three main mesenteric
arteries: the celiac artery (CA), the superior mesenteric artery (SMA), and the
inferior mesenteric artery (IMA) (Fig. 49.1). The CA, arising from the infradiaphragmatic suprarenal abdominal aorta, provides circulation to the foregut
(esophagus to duodenum), hepatobiliary system, and the spleen. The SMA, arising
just distal on the suprarenal aorta to the CA, supplies the midgut (jejunum to
mid-transverse colon). The IMA, which originates from the left lateral portion of
the infrarenal aorta, supplies the hindgut (mid-transverse colon to rectum). The
superior and inferior pancreaticoduodenal arteries provide collateral perfusion
between the CA and SMA, while the marginal artery of Drummond, arc of Riolan,
S. Parascandola (&) V. Obias
Department of Colorectal Surgery, George Washington University Medical Faculty Associates,
Washington, DC, US
e-mail: salvatore.parascandola@gmail.com
© 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_49
489
490 S. Parascandola and V. Obias
and retroperitoneal meandering arteries form collateral networks between the SMA
and IMA. Due to this redundancy in circulation, chronic reduction of flow in one or
two mesenteric vessels is typically tolerated, provided sufficient temporal compensatory perfusion by the uninvolved branches. Acute occlusion a main mesenteric
trunk, by contrast, can produce profound ischemia and necrosis in the absence of
collateral perfusion. Prompt recognition and treatment before the onset of irreversible ischemia are critical.
In embolic occlusion, emboli lodge at branch points distal to the middle colic
artery and early jejunal branches, sparing the SMA origin (Fig. 49.2). Risk factors
for those with embolic occlusion of the mesenteric arterial circulation include atrial
fibrillation, recent myocardial infarction, mitral valve disease, mural thrombus, or
left ventricular aneurysm. In the absence of well-formed collateral perfusion,
embolization results in a pattern of ischemia that compromises the majority of small
bowel and ascending colon while sparing the proximal jejunum and distal transverse colon (Fig. 49.3a).
Thrombosis of the SMA is usually associated with systemic, pre-existing
atherosclerosis. Many of these patients have a history of chronic mesenteric
ischemia (CMI) including ‘food fear’, post-prandial pain, and weight loss. SMA
thrombosis is also seen in the setting of vasculitis, mesenteric dissection, or mycotic
aneurysm. Thrombosis typically occurs at the origins of visceral arteries, leading to
continuous intestinal involvement in the distribution of either the CA or SMA,
without sparing the proximal jejunum or colon (Fig. 49.3b).
Fig. 49.1 Sagittal CT
demonstrates the three major
arteries that supply the bowel,
celiac axis (asterisk), superior
mesenteric artery (white
arrow) and inferior mesenteric
artery (orange arrow). All are
visceral branches of the
abdominal aorta (+)
49 Mesenteric Artery Thrombosis and Embolism 491
Fig. 49.2 Sagittal CTA scan
of a patient with acute
mesenteric ischemia
secondary to embolic
occlusion of the SMA (arrow)
Presentation
Patients with acute mesenteric artery occlusion may present with significant
abdominal pain that is out of proportion to a benign abdominal exam. In these
patients, high level of suspicion is key to early diagnosis. Nausea, vomiting, and
diarrhea may follow the onset of abdominal pain. Fever, guarding, and abdominal
tenderness are late findings, typically associated with bowel infarction. Patients may
be tachycardic and demonstrate melena or heme positive stools. History of
arrhythmia, prosthetic heart values, recent myocardial infection, or prior
embolization to other arterial trees should raise the suspicion of mesenteric emboli
in patients with acute onset of abdominal pain.
Thrombotic mesenteric occlusion may also present with sudden onset of severe
midabdominal pain that is out of proportion to the physical findings. However,
unlike patients with acute embolic occlusion, these patients often have a history
consistent with chronic mesenteric ischemia, which manifests as postprandial
abdominal pain leading to food avoidance and significant weight loss. These
patients may also demonstrate other sequelae of diffuse atherosclerotic disease e.g.
coronary or peripheral artery disease.
492 S. Parascandola and V. Obias
Fig. 49.3 a Embolic occlusion of the superior mesenteric artery with jejunal-sparing ischemia.
b Mesenteric arterial thrombosis with continuous intestinal involvement in the distribution of the
SMA, without sparing the proximal jejunum or colon. Reused with permission. Copyright ©
Springer Nature
Laboratory Findings
Although laboratory findings are often insensitive and nonspecific for the diagnosis
of mesenteric artery embolism or thrombosis, though they can help substantiate
clinical suspicion. The majority of patients will have an abnormally elevated
leukocyte count. Metabolic acidosis with elevated lactate develops as a result of
anaerobic metabolism. While an elevated lactate can be seen in the setting of
decreased oral intake and dehydration, a lactate level of >2 mmol/l is associated
with irreversible intestinal ischemia. Additionally, elevated D-dimer is associated
49 Mesenteric Artery Thrombosis and Embolism 493
with intestinal ischemia, reflecting ongoing clot formation and degradation by fibrinolysis. Elevated serum amylase and aspartate aminotransferase may also be seen.
Imaging Workup
Plain abdominal radiographs are typically the first test ordered in a patient with
acute abdominal pain. While they are helpful in ruling out other causes of
abdominal pain, they have a limited role in diagnosing acute mesenteric ischemia.
Plain films may demonstrate ileus and vascular calcifications, suggesting ischemia.
Such findings as bowel wall edema (thumbprinting), pneumatosis, pneumobilia, or
pneumoperitoneum may indicate infarction. Importantly, a negative radiograph
does not exclude acute mesenteric ischemia, as up to 25% of abdominal radiographs
will be normal.
Today, mul ti-detector CTA represents the most useful and rapid tool for the fast
and accurate diagnosis of AMI. In the presence of irreversible ischemia, CTA will
demonstrate intestinal dilatation and thickness, reduction or absence of visceral
enhancement, pneumatosis intestinalis, portal venous gas, or free intraperitoneal air
(Fig. 49.4a and b). Biphasic CTA is the diagnostic modality of choice and includes
the following steps: (1) Pre-contrast scans to detect hyper attenuating vascular
calcification, intravascular thrombus, and intramural hemorrhage, (2) arterial and
venous phases to demonstrate thrombus in the mesenteric arteries and veins,
presence of embolism or infarction of other organs, and abnormal enhancement of
the bowel wall, and (3) multi-planar reconstructions (MPR) to assess the origin of
the mesenteric arteries. Three-dimensional reconstructions generated from CTA
datasets can also provide valuable guidance for preoperative planning (Fig. 49.5).
For the diagnosis of AMI, a sensitivity of 93%, specificity of 100%, and positive
Fig. 49.4 CTA of the abdomen in a patient with embolism to the superior mesenteric artery
demonstrating a pronounced intrahepatic portal venous gas (red arrow) and b dilated, gas-filled
loops of bowel and transmural necrosis (orange arrow). Pneumatosis (white arrow), mesenteric
venous gas (white arrowhead), and fat stranding (asterisk) are seen
494 S. Parascandola and V. Obias
Fig. 49.5 3D CTA
reconstruction demonstrating
mid occlusion of SMA
(arrow)
and negative predictive values of 100 and 94%, respectively, have been achieved
with this imaging modality.
Catheter-based angiography is reserved for cases where the diagnosis is not
clear, endovascular interventions are contemplated, or further details of SMA
anatomy are required for SMA revascularization. Multiple views are needed for the
sufficient evaluation of the SMA and CA. Lateral views are used to assess the origin
of the CA and the SMA, while anteroposterior views allow for the evaluation of the
circulation of the distal CA and SMA. Selective catheterization of the SMA may
reveal a ‘meniscus sign’ at the branch points where emboli lodge.
Gadolinium-enhanced magnetic resonance angiography (MRA) avoids the risk
of allergic reaction and nephrotoxicity associated with iodinated CT imaging.
While MRA is helpful in diagnosing chronic mesenteric ischemia, in the setting of
acute ischemia, MRA and post processing are too time consuming for these critically ill patients. Given the consequences of a missed or delayed diagnosis, CTA
should be considered over MRA even in the setting of renal insufficiency.
Duplex ultrasonography has little value in the diagnosis of AMI. However, it is a
valuable noninvasive adjunct for follow up after surgical or endovascular revascularization and can be used to assess patency and recurrence of disease. Endoscopy, colonoscopy, or barium radiography offer no useful information in the
diagnostic evalua tion of acute mesenteric ischemia. Furthermore, if mesenteric
ischemia is considered, a barium enema is contraindicated as intraperitoneal
extravasation of barium can occur in the setting of intestinal perforation.