Rare extra-adrenal paraganglioma mimicking a painful Schwannoma

Rare extra-adrenal paraganglioma mimicking
a painful Schwannoma
Ann. Ital. Chir.
Published online (EP) 26 September 2014
pii: S2239253X14022907
www.annitalchir.com
Case report
PR RE
IN AD
TI -O
N
G NL
PR Y
O CO
H P
IB Y
IT
ED
Andrea Picchetto, Alessandro M. Paganini, Andrea Balla, Silvia Quaresima, Vito Cantisani,
Giancarlo D’Ambrosio, Emanuele Lezoche
Department of General Surgery, Surgical Specialties and Organ Transplantation “Paride Stefanini” (Direttore Prof. Emanuele Lezoche)
“Sapienza” University of Rome
U.O.C. Clinica Chirurgica e Tecnologie Avanzate
Rare extra-adrenal paraganglioma mimicking a painful Schwannoma. Case report
INTRODUCTION: Paragangliomas originate from chromaffin tissue primarily found in the Zuckerkandl body. A 53 years
old man, was investigated with abdominal MRI for chronic backache, that had lasted for 2 years.
CASE REPORT: MRI revealed an incidental mass in para-aortic region, at the adrenal lodge, with characteristics of a solid nodular mass, which did not seem to arise from the adrenal gland. An abdominal CT showed a mass localized prior to left adrenal gland; the radiologist reported this mass as a malignant peripheral nerve sheath tumor (Schwannoma).
During his stay in hospital, tumoral markers chromogranin and neuron-specific enolase were dosed, with a concentration of 187.00 ng/mL and 7.7 ng/mL. Patient’s back pain was treated first with ketorolac, without any resolution of
the problem, then with ketorolac plus tramadol in elastomeric pump, again with no relief, and finally with a continuous infusion of morphine. Since the exact nature of the neoplasm was not known and a CT guided biopsy was not
technically feasible to do, a laparoscopic excision of the mass, with transperitoneal anterior submesocolic approach, as well
as for the left adrenalectomy, was planned. After surgery, the patient no longer needed morphine. The postoperative course
was uneventful.
RESULTS: Histopathological findings consisted of periadrenal paraganglioma. Extra-adrenal paragangliomas are rarely recognized during preoperative study. This is often due to its capacity of mimicking other type of tumors.
CONCLUSIONS: Paragangliomas could be successfully resected by laparoscopy, they are difficult to distinguish from other
tumor and they should be investigated even if preoperative findings argue for something else.
KEY
WORDS:
Laparoscopic adrenalectomy, Paraganglioma, Schwannoma
Introduction
The incidence of paragangliomas is 1/100.000, and are
approximately ten percent of catecholamine secreting
tumors 1. Paragangliomas originate from chromaffin tissue
primarily found in the Zuckerkandl body, the kidneys, the
sympathetic plexus of the urinary bladder, and the heart
or in the sympathetic ganglia of the head or neck 1.
Due to the possibility of catecholamine’s secretion,
patients with paragangliomas may present with signs and
symptoms including hypertension, palpitations, tremor,
pallor, headache and tachyarrhythmia 2,10.
Catecholamine-secreting paragangliomas are only 25%.
The remainder present with an abdominal mass only and
no evidence of hypertension 3,10.
Case report
Pervenuto in Redazione Aprile 2014. Accettato per la pubblicazione
Maggio 2014
Correspondence to: Dott. Andrea Picchetto, Clinica Chirurgica e Tecnologie
Avanzate, Dept. “Paride Stefanini”, Policlinico “Umberto I”, Viale del
Policlinico 155, 00161 Roma (E-mail: andreapicchetto@gmail.com)
A 53 years old man, followed up for a previously treated Hodgkin’s lymphoma in 1993 and with hypercholesterolemia/hypertriglyceridemia, was investigated with
Published online (EP) 26 September 2014 - Ann. Ital. Chir
1
A. Picchetto, et al.
abdominal MRI for chronic backache that had lasted for
two years. The patient tried to relieve the back pain
(VAS Score 8), unsuccessfully, with several types of nonsteroideal anti-inflammatory drugs (NSAID). MRI
revealed an incidental para-aortic solid nodular mass
(29x25mm) located within the left adrenal lodge, which
did not seem to arise from the left adrenal gland. CT
scan (Fig. 2) showed the mass to be localized anteriorly to the left adrenal gland and was reported as a malignant peripheral nerve sheath tumor (Schwannoma) or,
alternatively, as a partially colliquated lymph node.
Biopsy, due to its difficult location, was not performed.
The patient did not report headaches, excessive sweating, palpitations, hypertension, or any other systemic
symptom. During his stay in hospital, tumor markers
chromogranin and neuron-specific enolase (NSE) were
187.00 ng/mL (n.v. <90.00 ng/mL) and 7.7 ng/mL (n.v.
<12 ng/mL), respectively.
The patient’s back pain was treated with ketorolac first,
with no relief, then with ketorolac plus tramadol in elastomeric pump, again with no relief, and finally with a
continuous infusion of morphine, which was successfully.
Treatment
Fig. 1: Trocar positions during left transperitoneal adrenalectomy.
The surgeon and first assistant stand on the left of the patient while
the second assistant stands on the right.
Fig. 2: Axial CT scan showed the presence of round shaped, well
marginated, hypodense lesions of....mm x mm. The lesion was strictly adjacent to adrenal gland.
PR RE
IN AD
TI -O
N
G NL
PR Y
O CO
H P
IB Y
IT
ED
Since the exact nature of the neoplasm was not known and
a CT guided biopsy was not technically feasible, a laparoscopic excision of the mass was planned, with a transperitoneal anterior submesocolic approach, as commonly performed by the authors for left adrenalectomy 4-5.
Surgery was performed under general anesthesia. A nasogastric tube and a urinary catheter were positioned. The
patients was monitored with an intra-arterial radial artery
catheter for blood pressure measurement and a central
venous catheter (internal jugular access) to monitor
changes in central venous pressure and for rapid introduction of liquids, if necessary. Pneumoperitoneum was
established with a Veress needle at the umbilicus.
Pneumoperitoneum at 13 mmHg pressure was obtained
with carbon dioxide insufflator adjusted at 30 l/minute.
Four 12mm trocars (Fig. 1) and a 45° optic were used.
This approach was performed with the patient supine,
in anti-Trendelenburg position and with the operating
table rotated 30° towards the opposite side of the lesion,
to facilitate exposure of the surgical field. The surgeon
stands on the side ipsilateral to the lesion.
The first trocar was inserted to the left of the midline
above the umbilicus (Fig. 1). A second trocar was inserted on the right midclavicular line below the right costal
arch. A third and fourth trocar were placed one on the
left midclavicular line above the transverse umbilical line
and one on the left anterior axillary line, respectively.
The transverse mesocolon was raised by the assistant with
atraumatic forceps introduced through the right midclavicular trocar to expose the first jejunal loop at the
ligament of Treitz and to identify the inferior mesenteric
vein. The posterior peritoneum was opened at the insertion of the transverse mesocolon on the lower edge of
2
Ann. Ital. Chir - Published online (EP) 26 September 2014
Rare extra-adrenal paraganglioma mimicking a painful Schwannoma. Case report
icked a malignant peripheral nerve sheath tumor, a
MIBG scintigraphy, however, was not performed.
Paragangliomas are difficult to distinguish from other
tumors. Any extra-adrenal mass should be investigated
even if the preoperative findings argue for something
else.
In this patient, the paraganglioma mimicked an adrenal
mass and it was treated with the same laparoscopic
approach that is commonly employed by the authors to
treat adrenal masses, with good results. The submesocolic approach was found to be safe and feasible to treat
this peri-adrenal mass.
PR RE
IN AD
TI -O
N
G NL
PR Y
O CO
H P
IB Y
IT
ED
the pancreas, between the first jejunal loop and the inferior mesenteric vein. After Toldt’s fascia identification,
the dissection proceeded posteriorly along the retro-pancreatic plane, elevating the body of the pancreas. Then
Gerota’s fascia was opened to identify the superior margin of the left renal vein. This was prepared latero-medially until its junction with the inferior adrenal vein,
which was adferent the mass. The left adrenal vein was
prepared and divided between clips (AcuClip, Tyco /
Healthcare, Norwalk, Connecticut, USA) to allow access
to the mass. After adrenal gland division, the medial
subdiaphragmatic connections of the mass with the surrounding anatomical structures were divided with a
radiofrequency vessel sealing device (LigaSure ™ tissue
fusion, Covidien, Mansfield, Massachusetts, USA).
Monopolar electrocautery was selectively used for hemostasis. Once released, the mass was removed inside an
extraction bag. The residual space was filled with hemostatic material (Floseal, Baxter Healthcare Corporation,
Deerfield, Illinois, USA) and a drainage was left in place.
After surgery, the patient no longer needed morphine
and the pain was easily controlled with paracetamol, suspended on POD 3. The postoperative course was
uneventful.
The resected specimen consisted of a brownish tissue of
5x3x2 cm, with an area of central colliquation.
Histological examination revealed a polygonal cells’ nests
mass, which presented a round- to oval-cell predominant
smear with occasional striking anisonucleosis, free of histological anomalies. Mitosis or necrotic areas were not
seen. Immunohistochemical stainings have reported diffuse positivity with chromogranin, synaptophysin and
S100 protein; CKAE1-AE3 and melan-A were negative.
The proliferation index was found to be 2-3%.
Histopathological findings were consistent with the diagnosis of periadrenal paraganglioma.
Follow-up
The patient is alive, free of symptoms and has been followed up for 15 months. Follow up abdominal CT and
MRI scans were repeated, both of them were unremarkable.
Discussion/Conclusion
Extra-adrenal paragangliomas are rarely recognized preoperatively. This is often due to its capacity of mimicking other types of tumors 6-15. Magnetic resonance
imaging (MRI and computerized tomography (CT) are
highly sensitive in detection of small tumors but are not
specific for paragangliomas 16-17. MIBG scintigraphy is
specific for paraganglioma but is less sensitive than MRI
and CT 18. Due to normal plasma and urine catecholamine levels and to CT and MRI findings that mim-
Riassunto
I paragangliomi hanno un’incidenza annuale di circa
1/100000; essi originano dai tessuti cromaffini dei corpi di Zurckerkandl.
In questo case report riportiamo il caso di un uomo di
53 anni, già trattato e seguito per un precedente limfoma di Hodkin che è stato sottoposta a RMN dell’addome
per una lombalgia cronica che durava da due anni e che
non riusciva ad alleviare con i FANS.
La RMN ha rivelato un’incidentaloma: una massa in
regione para-aortica, a livello della loggia surrenalica sinistra con le caratteristiche di una massa nodulare solida
(29*25mm), che non sembrava originare direttamente dal
surrene. Il pz ha inoltre eseguito un TC dell’addome che
ha mostrato una massa localizzata di fronte al surrene di
sinistra; il collega radiologo ha definito questa massa
come un tumore maligno delle guaine nervose periferiche (Schwannoma) o come un linfonodo colliquato
(essendo nota la patologia linfomatosa già descritta in
anamnesi). Il paziente non riferiva cefalea, palpitazioni,
picchi ipertensivi ne’ altri sintomi sistemici.
Durante il suo ricovero, sono stati dosati i marker tumorali cromogranina e l’enolasi neurono-specifica che sono
risultati essere rispettivamente 187.00 ng/mL and 7.7
ng/mL.
La lombalgia è stata trattata inizialmente con Ketorolac,
senza beneficio alcuno, quindi con Ketorolac più
Tramadolo in pompa elastomerica, nuovamente senza
beneficio per il paziente, infine con infusione continua
di Morfina. Dato che l’esatta natura della neoformazione non era nota e che l’esecuzione di una biopsia TC
guidata non era tecnicamente possibile, è stata eseguita
un’escissione per via laparoscopica della massa, con
approccio anteriore submesocolico trans peritoneale, così
come gli Autori sono soliti eseguire per la surrenalectomia sinistra. Sin dai primi giorni postoperatori il paziente non ha più necessitato della morfina. Il decorso postoperatorio è stato del tutto regolare. L’esame istologico è
risultato essere paraganglioma. I paragangliomi extra-surrenalici sono raramente riconosciuti duranti gli esami
preoperatori. Questo errore è spesso dovuto alla sua capacità di simulare vari altri tipi di tumore.
Published online (EP) 26 September 2014 - Ann. Ital. Chir
3
A. Picchetto, et al.
Gli Autori credono che i paragangliomi possano essere
resecati con successo tramite approccio laparoscopico, che
essi sono difficili da distinguere altri tipi di tumore e
che debbano essere sospettati anche se i risultati preoperatori depongono per qualcos’altro.
References
1. Plouin PF, Gimenez-Roqueplo AP: Pheochromocytomas and
secreting paragangliomas. Orphanet J Rare Dis, 2006; 1:49.
10. Giangrande DL, Paliogiannis P, Denti S, Attene F, Scognamillo
F, Serventi F, Trignano M: Surgical management of a voluminous
abdominal paraganglioma and review of the literature. Ann Ital Chir,
www.annitalchir.com, 2011; 82(3):211-15.
11. Capel I, Gil MP, Marqués G, Barcons S, Rigla M: Cervical
paraganglioma mimicking a thyroid nodule with recurrent
nerve involvement. Endocrinol Nutr, 2012; 59(4):274-5. doi:
10.1016/j.endonu.2011.09.011. Epub 2011 Dec 11. Spanish.
12. Durai R, Husain F, Oke T, Siddiqui M: Paraganglioma mimicking adrenal incidentaloma. Br J Hosp Med (Lond), 2011;
72(10):593.
PR RE
IN AD
TI -O
N
G NL
PR Y
O CO
H P
IB Y
IT
ED
2. Young WF Jr.: Paragangliomas: clinical overview. Ann NY Acad
Sci, 2006; 1073:21-9.
9. Bartolacci ML, Bartolacci S, Leombruni E, Annunziata A, Di
Rienzo M, Picardi N: Adrenal incidentaloma: a dopamine-secreting
pheochromocytoma with an atypical clinical picture. A case report and
review of the literature. Ann Ital Chir, www.annitalchir.com, 1998;
69(6):805-14.
3. Muzaffar S, Fatima S, Siddiqui MS, Kayani N, Pervez S Raja
AJ: Mesenteric paraganglioma. Can J Surg, 2002; 45(6):459-60.
4. Scoglio D, Balla A, Paci M, Guerrieri M, Lezoche G,
D’Ambrosio G, Fabiani B, Ursi P, Paganini AM: Laparoscopic transperitoneal anterior adrenalectomy. Ann Ital Chir, 2013; 84:411-16.
5. Perretta S, Campagnacci R, Guerrieri M, Paganini AM, De
Sanctis A, Sarnari J, Rimini M, Lezoche E: Sub-mesocolic access in
laparoscopic left adrenalectomy. Surg Endosc, 2005; 19(7):977-80.
6. Amin MF, EL Ameen NF: Diagnostic efficiency of multidetector
computed tomography versus magnetic resonance imaging in differentiation of head and neck paragangliomas from other mimicking vascular lesions: Comparison with histopathologic examination. Eur Arch
Otorhinolaryngol, 2013; 270(3):1045-53. doi: 10.1007/s00405-0122084-6. Epub 2012 Jun 27.
7. Lee HM, Jeong DS, Park PW, Kim WS, Sung K, Lee YT:
Anterior mediastinal paraganglioma mimicking thymoma. Heart Surg
Forum, 2012; 15(3):E170-1. doi: 10.1532/HSF98.20111173.
8. Napolitano L, Francomano F, Angelucci D, Napolitano AM:
Thyroid paraganglioma: Report of a case and review of the literature.
Ann Ital Chir, www.annitalchir.com, 2000; 71(4):511-13.
4
Ann. Ital. Chir - Published online (EP) 26 September 2014
13. Singhi AD, Hruban RH, Fabre M, Imura J, Schulick R,
Wolfgang C, Ali SZ: Peripancreatic paraganglioma: a potential diagnostic challenge in cytopathology and surgical pathology. Am J Surg
Pathol, 2011; 35(10):1498-504.
14. Tuncel A, Aslan Y, Han O, Horasanli E, Seckin S, Atan A:
Laparoscopic resection of periadrenal paraganglioma mimicking an isolated adrenal hydatid cyst. JSLS, 2010; 14(4):579-82.
15. Lightfoot N, Santos P, Nikfarjam M: Paraganglioma mimicking
a pancreatic neoplasm. JOP, 2011; 12(3):259-61.
16. Sahdev, Sohaib A, Monson JP, Grossman AB, Chew SL, Reznek
RH: CT and MR imaging of unusual locations of extra-adrenal paragangliomas (pheochromocytomas). Eur Radiol, 2005; 15:85-92.
17. Chen H, Sippel RS, O’Dorisio MS, Vinik AI, Lloyd RV, Pacak
K: The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: Pheochromocytoma, paraganglioma,
and medullary thyroid cancer. Pancreas, 2010; 39(6):775-83.
18. Whitson BA, Tuttle TM: Laparoscopic resection of periaortic paragangliomas. Am Surg, 2005; 71:450-454. Pancreas. 2010; 39(6):775-83.

Download Report