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J. Otto et al.
– Classication of the technique accord-
ing to mesh positioning: epifascial
(onlay), retromuscular (sublay) intraperitoneal mesh reinforcement
(. Fig.13.4).
Minimally Invasive Procedures
5 Laparoscopic hernia reduction + intraper-
itoneal mesh reinforcement.
5 Techniques:
– IPST mesh (intraperitoneal stoma
mesh): Mesh with a central tunnel (from
2–3.5cm diameter) is placed around the
terminal stoma and xed to the abdominal wall.
– Sugarbaker technique: reduction of the
hernia + lateralization of the executing
intestinal loop with a covering mesh.
– Sandwich technique: reduction of the
hernia + positioning of a keyholeshaped mesh around the terminal intestinal loop + additional lateralization of
this loop with another mesh in the sense
of the Sugarbaker technique.
5 Hernial gap: between the aponeurosis of
the internal oblique muscle and the outer
edge of the rectus sheath (linea semilunaris).
13.7.2 Hernia Obturatoria
5 Hernia of the pelvic oor (. Fig.13.5).
5 Hernial gap in the obturator foramen,
along the vasa obturatoria/N. obturatorius.
5 Especially for older women.
5 Clinical presentation: Typically pain in the
lower abdomen + radiation to the inner
thighs.
5 Caution: Not visible externally.
13.7.3 Hernia Ischiadica
5 Hernial gap=foramen ischiadicum in the
region of the gluteus maximus muscle
(. Fig.13.5).
13
13.6.7 Guidelines
Bittner R, Bingener-Casey J, Dietz U, Fabian
M, Ferzli GS, Fortelny RH, Köckerling F,
Kukleta J, Leblanc K, Lomanto D, Misra
MC, Bansal VK, Morales-Conde S, Ramshaw
B, Reinpold W, Rim S, Rohr M, Schrittwieser
R, Simon T, Smietanski M, Stechemesser B,
Timoney M, Chowbey P, IEHS (2014)
Guidelines for laparoscopic treatment of ventral and incisional abdominal wall hernias.
International Endohernia Society (IEHS) –
Part 3. Surg Endosc 28: 380–404. 7 https://
doi.org/10.1007/s00464- 01m3- 3172- 4
13.7 Other Rare Hernias
13.7.1 Spieghel’s Hernia (Hernia
Lineae Semilunaris)
5 Hernia form in the lower middle abdomen.
13.7.4 Hernia Perinealis
5 Hernia through ischiorectal fossa
(. Fig.13.5).
5 Course: to the perineum or into the labia
majora.
13.7.5 Lumbar Hernia
5 Hernia in the region of the upper (Trigo-
num lumbale superius)/lower lumbar triangle (Trigonum lumbale inferius).
5 Trigonum lumbale = weak point of the
lumbar abdominal wall.
5 Trigonum lumbale superius: triangle
between lateral border of the Mm. erector
spinae and medial border of the M.
obliquus internus, below the 12th rib.
5 Trigonum lumbale inferius (Petit):
between edges of the M. obliquus externus and latissimus dorsi, above the iliac
crest.
Hernia
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321
13
paravesical hernia
retrovesical hernia
ischiorectal hernia
spinotuberous hernia
infrapiriform hernia
suprapiriform hernia
. Fig. 13.5 Rare hernias of the pelvic oor
References
European Hernia Society guidelines (2009) on the treat-
ment of inguinal hernia in adult patients. Hernia
13:343–403. https://doi.org/10.1007/s10029- 009-
0529- 7
Schumpelick V (ed) (2011) Gastroenterologische
Chirurgie, 3rd edn. Springer, Berlin/Heidelberg
Further Reading
Bakker WJ, Aufenacker TJ, Boschman JS, Burgmans
JPJ (2020) Lightweight mesh is recommended in
open inguinal (Lichtenstein) hernia repair: a sys-
tematic review and meta-analysis. Surgery 167:
581–589
Bernardi K, Olavarria OA, Holihan JL, Kao LS, Ko TC,
Roth JS, Tsuda S, Vaziri K, Liang MK (2019)
Primary fascial closure during laparoscopic ventral
hernia repair improves patient quality of life: a mul-
ticenter, blinded randomized controlled trial. Ann
Surg 271:434–439
Blonk L, Civil YA, Kaufmann R, Ket JCF, van der Velde
S (2019) A systematic review on surgical treatment of
primary epigastric hernias. Hernia 23:847–857
Bökkerink WJV, Koning GG, Malagic D, van Hout L,
van Laarhoven CJHM, Vriens PWHE (2019) Long-
term results from a randomized comparison of
open transinguinal preperitoneal hernia repair and
the Lichtenstein method (TULIP trial). Br J Surg
106:856–861
obturator hernia
puborectalis muscle
anal canal hiatus
obturatorius internus
muscle
lesser sciatic foramen
greater sciatic foramen
coccygeus muscle
pubococcygeus muscle
Bullen NL, Massey LH, Antoniou SA, Smart NJ,
Fortelny RH (2019) Open versus laparoscopic mesh
repair of primary unilateral uncomplicated inguinal
hernia: a systematic review with meta-analysis and
trial sequential analysis. Hernia 23:461–472
Caro-Tarrago A, Olona C, Millán M, Olona M, Espina
B, Jorba R (2019) Long-term results of a prospective randomized trial of midline laparotomy closure
with onlay mesh. Hernia 23:335–340
Gavriilidis P, Davies RJ, Wheeler J, de’ Angelis N, Di
Saverio S (2019) Total extraperitoneal endoscopic
hernioplasty (TEP) versus Lichtenstein hernioplasty: a systematic review by updated traditional
and cumulative meta-analysis of randomisedcontrolled trials. Hernia 23:1093–1103
Gutlic N, Gutlic A, Petersson U, Rogmark P,
Montgomery A (2019) Randomized clinical trial
comparing total extraperitoneal with Lichtenstein
inguinal hernia repair (TEPLICH trial). Br J Surg
106:845–855
van den Hil LCL, van Steensel S, Schreinemacher MHF,
Bouvy ND (2019) Prophylactic mesh placement to
avoid incisional hernias after stoma reversal: a systematic review and meta-analysis. Hernia 23:
733–741
Kohler A, Lavanchy JL, Lenoir U, Kurmann A,
Candinas D, Beldi G (2019) Effectiveness of prophylactic intraperitoneal mesh implantation for prevention of incisional hernia in patients undergoing
open abdominal surgery: a randomized clinical
trial. JAMA Surg 154:109–115
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Lockhart K, Dunn D, Teo S, Ng JY, Dhillon M, Teo E,
van Driel ML (2018) Mesh versus non-mesh for
inguinal and femoral hernia repair. Cochrane
Database Syst Rev 9:CD011517
Obermaier R, Pfeffer F, Hopt UT (eds) (2009)
Hernienchirurgie. Elsevier Urban & Fischer, München
Patterson TJ, Beck J, Currie PJ, Spence RAJ, Spence G
(2019) Meta-analysis of patient-reported outcomes
after laparoscopic versus open inguinal hernia
repair. Br J Surg 106:824–836
Sheen AJ, Montgomery A, Simon T, Ilves I, Paajanen H
(2019) Randomized clinical trial of open suture
repair versus totally extraperitoneal repair for treatment of sportsman’s hernia. Br J Surg 106:837–844
Shrestha D, Shrestha A, Shrestha B (2019) Open mesh
versus suture repair of umbilical hernia: metaanalysis of randomized controlled trials. Int J Surg
62:62–66
Berger D (2010) Laparoskopische reparation der para-
stomalen Hernie. Chirurg 81:988–992
Jones HG, Rees M, Aboumarzouk OM, Brown J, Cragg
J, Billings P, Carter B, Chandran P (2018) Prosthetic
mesh placement for the prevention of parastomal
herniation. Cochrane Database Syst Rev
7:CD008905
Reinforcement of Closure of Stoma Site (ROCSS),
Collaborative and West Midlands Research
Collaborative (2020) Prophylactic biological mesh
reinforcement versus standard closure of stoma site
(ROCSS): a multicentre, randomised controlled
trial. Lancet 395:417–426
Rosch R, Conze J, Junge K, Neumann U (2010)
Konventionelle Reparation der parastomalen
Hernie. Chirurg 81:982–987
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Gastrointestinal Stromal
Tumors andSarcomas
DanielOertli, HolgerBannasch, AthanasiosTampakis,
ChristophKettelhack, andTobiasKeck
Contents
14.1 Gastrointestinal Stromal Tumours (GIST) – 325
14.1.1 Denition – 325
14.1.2 Epidemiology andTumour Localisation – 325
14.1.3 Clinical Presentation – 325
14.1.4 Pathology – 326
14.1.5 Diagnosis – 326
14.1.6 Therapy – 326
14.1.7 Guidelines – 328
14
14.2 Soft Tissue Tumours oftheExtremities – 328
14.2.1 General: Classication – 328
14.2.2 Clinical Presentation – 329
14.2.3 Diagnosis – 330
14.2.4 Classication – 331
14.2.5 Prognosis – 332
14.2.6 Therapeutic Principles – 332
14.2.7 Guidelines – 335
14.3 Retroperitoneal Sarcomas – 335
14.3.1 Epidemiology andPrognosis – 335
14.3.2 Pathology – 335
14.3.3 Classication – 336
© The Author(s), under exclusive license to Springer-Verlag GmbH, DE, part of Springer
Nature 2023
F. Billmann, T. Keck (eds.), Essentials of Visceral Surgery,
https://doi.org/10.1007/978-3-662-66735-4_14
14.3.4 Molecular Genetics – 336
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14.3.5 Clinical Presentation – 336
14.3.6 Diagnosis – 337
14.3.7 Therapy – 337
14.3.8 Management ofRecurrences – 339
14.3.9 Follow up – 339
14.3.10 Guidelines – 339
References – 339
Gastrointestinal Stromal Tumors andSarcomas
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325
14
14.1 Gastrointestinal Stromal
Tumours (GIST)
D.Oertli and T.Keck
Key Points
5 GIST = Most common soft tissue
tumors in the gastrointestinal tract.
5 Overexpression of the KIT tyrosine
kinase receptor (CD 117):
– Diagnostic marker.
– Simultaneous target for systemic
therapy.
5 Smaller tumors consistently benign;
with larger diameter = malignant
potential (occasionally liver metastases).
5 Radical surgical excision indicated
(goal = R0 resection)= avoidance of
local recurrences.
5 Adjuvant/neoadjuvant antibody ther-
apy: with imatinib (“rst line”)/with
sunitinib (“second line”).
Localization
5 Stomach: 60–70%.
5 Small intestine: 20–30.
5 Colorectum: <5%.
5 Other localization: esophagus, omentum,
mesentery <5%.
5 Most GIST=sporadic.
5 Solitary in 95% of cases.
Tumor Biology
5 Locally displacing growth.
5 Tumor originates from the tunica muscu-
laris.
5 Usually no lymphatic metastasis.
5 Hematogenous or peritoneal metastasis
possible, 20% of tumors are metastatic at
diagnosis.
Risk Factors
5 Neurobromatosis.
5 In the context of the Carney triad:
extraadrenal paraganglioma, pulmonary
chondroma, GIST.
5 Familial accumulation possible.
14.1.1 Denition
5 GIST (gastrointestinal stromal
tumors) = soft tissue tumors of the GI
tract.
5 Malignant potential dependent on:
– Differentiation (G=Grading).
– Tumor size.
– Metastasis.
14.1.2 Epidemiology andTumour
Localisation
Epidemiology
5 Annual incidence = 1/100,000 in general
population.
5 Women: Men=1: 1.
5 Peak incidence=5th–7th decade of life.
14.1.3 Clinical Presentation
Manifestation
5 70% symptomatic.
5 20% Incidental nding during endoscopy
or surgery.
5 10% autopsy nding.
Symptoms
5 Often incidental nding.
5 Gastrointestinal bleeding 52%; if localized
in the stomach: bleeding in 70% of cases.
5 Abdominal pain 32%.
Prognosis at Diagnosis
5 5-year survival of the total collective
(SEER database)=45%.
– Primary tumor = 53% (5-year sur-
vival=64%).
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D. Oertli et al.
14
– Locoregional recurrence=19%.
– Stage IV (metastatic)=23% (5-year sur-
vival=13%).
14.1.4 Pathology
Conventional Histology
5 Homogeneous proliferation of mesenchy-
mal cells.
5 gastrointestinal tract: cells with character-
istic features of interstitial Cajal cells
(pacemaker cells for peristalsis located in
muscle wall with characteristic features of
both smooth muscle and autonomic nerve
cells).
5 Histological subtypes:
– Spindle cell type (70%).
– Epithelioid cell type (20%).
– Mixed type (spindle + epithelioid
cells=10%).
Immune Phenotype
5 KIT tyrosinase receptor (CD 117) overex-
pression: 95% of cases.
– CD 117=product of the c-KIT gene (=
protooncogene).
– Diagnostic marker in immunohisto-
chemistry.
– Most other mesenchymal tumors
(except Ewing’s sarcoma) = CD
117-negative.
5 Other positive markers:
– CD 34: 70%.
– “Smooth muscle actin” (SMA): 25%.
– S-100 <10%.
– Desmin <5%.
Mutation Detection
5 Mutation in KIT receptor / in platelet-
derived growth factor receptor α (PDGFRA): Functionally corresponding to
protooncogene.
5 Mutated protooncogene permanently
active: cell proliferation and tumor formation.
14.1.5 Diagnosis
Diagnostic Imaging
5 Primary diagnosis by esophagogastroduode-
noscopy (EGD), in case of tumor localization in the small intestine push/double
balloon endoscopy (advancing the endoscope into the small intestine with the help
of balloons) or capsule endoscopy necessary.
5 In the case of unclear tumour extension or
to exclude metastases, additional CT-scan.
5 For tumor search in the small intestine:
dynamic magnetic resonance examination
with oral contrast medium (MRI-Sellink)
useful.
5 Standard diagnosis CT, MRI and FDG
PET if necessary for therapy assessment.
Tissue Biopsy
5 Indications:
– If endoscopically accessible (± endolu-
minal sonography).
– If tumor is unresectable or metastatic.
– If neoadjuvant pretreatment is planned.
5 Contraindication/no indication: If tumour
appears to be primarily completely resectable.
! Caution
Tumor seeding on biopsy and/or postinterventional bleeding from tumor.
14.1.6 Therapy
Surgical Therapy
Indication
5 Surgery=standard therapy for resectable
GIST.
General Principles
5 Minimum distance to the tumour: of 1–2cm
sufcent under certain circumstances.
Gastrointestinal Stromal Tumors andSarcomas
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– Partial resection of the stomach possible
(e.g. wedge resection): Gastrectomy
necessary in rare cases.
– Segment resections of the small and
large intestine.
– Local R0 resection of the rectum per-
mitted.
5 No systematic lymphadenectomy (because
predominantly hematogenous metastasis).
! Caution
A complete microscopic resection (R0
resection) should always be aimed for!
GIST oftheStomach
5 Tumour diameter 0.3–6.5 cm = mostly
“low risk”.
5 Small tumors: well localizable + resectable
by laparoscopy +/intraoperative gastroscopy.
327
5 c-KIT mutation: Higher response rate
than wild type.
5 As a consequence 2 strategies possible:
– Pro: High dosage (800mg daily) in
wild type and in exon 9 mutation.
– Contra: 400 mg in all patients,
increase to 800mg if progression.
5 Side effects: Anemia, neutropenia,
fatigue, edema, rash, nausea, diarrhea.
Second-Line Therapy
5 In case of resistance to imatinib (Gleevec):
– 5% primary resistance.
– 14% Secondary resistance.
5 Sunitinib: Different clinical benet
depending on genotype: KIT exon 9: 63%;
wild type 56%; KIT exon 11: 36%; PDGFRA 25%.
14
! Caution
If tumor rupture (spontaneous, traumatic,
iatrogenic, intraoperative) and tumor cell
seeding:
5 Adjuvant therapy (see below).
5 Without imatinib high local recurrence
risk.
Adjuvant Treatment
Antibodies (Ab) Against theTyrosine
Kinase Receptor
5 Imatinib (e.g. Gleevec) = selective thyro-
sine kinase inhibitor on c-KIT and PDGFRA.
5 Central role of the c-KIT mutation status
(see below “Molecular genetic analysis”).
5 Dosage=400–800mg daily.
5 Median time to maximum response:
197days.
5 Results
– Complete remission=5%.
– Partial remission=47%.
– Disease stabilisation=32%.
Neoadjuvant Treatment
Goals
5 Achieving resectability in primary irrect-
able GIST.
– Tumor regression (size): Can function-
preserving surgery be facilitated? Can a
multivisceral resection be avoided?
– Minimization of tumor fragility.
5 Based on imatinib (Gleevec).
Indications/Target Organs
5 Locally advanced tumor.
5 Target Organs:
– Rectum.
– Esophagus.
– Pancreas.
Multimodal Therapy
5 Indication depends on the risk of recur-
rence; this can be assessed, for example,
with the score according to Fletcher etal.
(2002a, b) (. Table 14.1) or Miettinen
and Lasota (2001).
Molecular Genetic Analysis as a Guide for
Ab Treatment
5 c-KIT exon 11: mutation higher response
rate than c-KIT exon 9 mutation.
Results ofNeoadjuvant Therapy
5 Maximum tumor response after
6–12months: Surgery.
5 Despite R0 resection: local recurrence (up
to 76% of patients).
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D. Oertli et al.
. Table 14.1 Risk stratication according to
Fletcher in GIST
Risk Tumor
size
Very low <2cm <5/50HPF
Low 2–5cm <5/50HPF
Intermediary
High >5cm
HPF high power eld
<5cm
5–10cm
>10cm
Any size
Microscopy: number of
mitoses
6–10/50HPF
<5/50HPF
>5/50HPF
Each number
>10/50HPF
5 In this case salvage surgery: 15 months
median survival.
5 Prognostic factors (poor prognosis):
– Tumor size.
– Tumor localization (duodenum, rec-
tum).
– Incomplete resection.
– High proliferation rate (Ki-67 index).
– Mutation status (deletion in KIT
exon11).
Assessment oftheTherapeutic
Success inImaging
Positron Emission Tomography (PET)
5 Decrease in SUV (“standardized uptake
value”) by more than 40% from baseline or
SUV <3.4=response to neoadjuvant therapy.
14.1.7 Guidelines
ESMO (2018) Clinical Practice Guidelines for
diagnosis, treatment and follow-up. Ann
Oncol 29 (Suppl 4): iv 68–78.
14.2 Soft Tissue Tumours
oftheExtremities
H.Bannasch
14.2.1 General: Classication
Key Points
5 Benign soft tissue tumors 90% vs. Soft
tissue sarcomas 10%.
5 In 60% of cases=Extremities affected.
5 Diagnosis: Ultrasound, MRI, PET.
5 Prognosis: Depending on tumor size +
grading.
14
! Caution
Cross-sectional imaging needed to evaluate therapeutic success.
5 Absence of increase in size of tumor and
metastases=response.
5 Especially if tissue density changes under
Ab therapy.
Computer Tomography (CT scan)
5 Therapy success=no increase in size (not
necessarily shrinkage of the lesion).
5 Decrease in measured density (in Houn-
seld units) >15%=remission.
Epidemiology
General Information
5 Benign soft tissue tumors.
– Large majority of all soft tissue tumours
(at least 100 times more frequent than
soft tissue sarcomas).
– Incidence=approx. 300/100,000.
5 Soft tissue sarcomas (STS)
– Rare and heterogeneous group of
malignant tumors.
– Mesenchymal origin.
– STS=only 1% of all adult malignancies.
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14
– Incidence in adults = approx.
2–3/100,000.
– Incidence increase of STS in the last
decades (possibly only apparent increase
due to better data collection).
– No differences in the geographical fre-
quency distribution.
– In children: Proportion of STS in all
malignancies signicantly higher
(5–8%) despite lower incidence.
STS Body-Distribution Pattern
5 60% Extremities.
– Lower extremity more often than upper
extremity.
– In each case, higher incidence for the
proximal limb sections.
5 20–35% retro- and intraperitoneal sarco-
mas including GIST (7 Sect. 14.1).
5 15–20% trunk, head and neck region.
! Caution
Soft tissue sarcomas in adults vs. children:
signicant differences with regard to distribution pattern in the body, histological
frequency distribution of the subtypes and
tumor biology!
Rare Hereditary Risk Factors
5 Li-Fraumeni Syndrome.
– Very rare autosomal dominant disease
with germline mutation of the tumor
suppressor gene p53.
– Very high risk for numerous malignan-
cies.
5 Neurobromatosis type 1 (von Reckling-
hausen’s disease).
– Incidence approx. 35/100,000.
– Autosomal dominant inherited multior-
gan disease.
– Mainly involvement of the skin and
nervous system (phacomatosis).
– including benign neurobromas.
– Lifetime risk of about 5–10% for malig-
nant transformation of a (plexiform)
neurobroma into a malignant periph-
eral nerve sheath tumor (MPNST).
Acquired Risk Factors
5 Postradiogenic sarcomas (latency of up to
20years).
5 Steward-Treves syndrome (angiosarcoma
originating from chronic lymphedema).
5 Viral infections in immunocompromised
patients causally responsible for STS
development:
– HHV8 for Kaposi’s sarcoma.
– EBV for leiomyosarcoma.
! Caution
Etiology of soft tissue sarcomas mostly
unclear; rare hereditary and acquired risk
factors known.
14.2.2 Clinical Presentation
5 Leading symptom=mostly painless swell-
ing of the extremities.
5 Duration of symptoms often not ascer-
tainable.
5 Frequent anamnestic association with
minor trauma (patient’s need for causality).
5 Rarely specic symptoms with peripheral
functional decits– vascular-nervous (blood
ow, motor function, sensory function) decits often only in advanced local stages.
5 B-symptomatics only present in excep-
tional cases.
! Caution
Increased delay in diagnosis:
5 Due to the rarity of soft tissue sarcomas
(often the possibility of a malignant tumour
is not considered).
5 Diagnosis in many cases in the context of
an inadequate excision without prior diagnosis.
Anamnesis
5 Despite oligosymptomatic: careful history
and examination obligatory.
5 Documentation of extent and duration of
swelling/discomfort.
5 Querying risk factors (see above).
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