TUMEUR D’ABRIKOSSOF DES NERFS

DU MEMBRE SUPÉRIEUR

GRANULAR CELL NERVE TUMOR OF

THE UPPER LIMB

Christian Dumontier MD, PhD

Centre de la Main, Guadeloupe, FWI

www.diuchirurgiemain.org
 HISTORICAL
• 1st case reported by Weber in 1854 for a tumor of
the tongue

• Abrikossof (1926) named it

« myoblastenmyome » (myoblactic myoma)

• Granular cell tumor was introduced by Lack (1980)

Abrikossoff A (1926). Über Myome, ausgehend von der quergestreiften willkürlichen Muskulatur. Virchows Archiv für pathologische Anatomie
und Physiologie und für klinische Medizin, 260: 215–233.
Lack EE, Worsham GF, Callihan MD, Crawford BE, Klappenbach S, Rowden G, et al. Granular cell tumor: a clinicopathologic study of 110
patients. J Surg Oncol 1980;13:301–316.
Weber CO: Anatomische Untersuchung einer hypertrophischen Zunge nebst Bemerkungen fiber die Neubildung quergestreJfter Muskel fasern.
Virchows Arch Pathol Anal 7:115-125, 1854.
 GRANULAR CELL TUMOR
• Of Neural derivation

• Granular appearance is due to the accumulation of

secondary lysosomes in the cytoplasm

• Granular cell tumors are typically solitary, smaller than

3 cm, and located in the dermis or subcutis and less
frequently in the submucosa, smooth muscle, or
striated muscle. May be multiple (in blacks ?)
 GRANULAR CELL TUMOR
• Granular cell tumors are mostly found in the
internal organs, particularly in the upper
aerodigestive tract (Tongue +++).

• Benign (98%) and malignant (1-2%)

• Incidence 0,017 to 0,029% of all pathological

specimens

• More common in females (2/1) and in middle

age patient (40-60 yrs old)

• Predominance in black people (74 and 82%)

has been reported
 • Granular cell tumors are uncommon
and composed of cells with
eosinophilic, granular cytoplasm caused
by an abundance of lysosomes filled
with periodic acid–Schiff-positive but
diastase-resistant material

• Granular cell tumors are generally

accepted to have neural differentiation
because they have been documented
to arise in association with small
peripheral nerves, stain uniformly for
S100 protein, and have ultrastructural
features suggesting neural
differentiation

Fisher KW, Hattab EM. Nerve tumors. in Nerves and nerve injuries, vol 2. Pain, treatment, injury, disease, and future directions. Tubbs RS, Rizk E,
Shoja MM, Loukas M, Barbaro N, Spinner RJ (eds). Elsevier 2015
 • When associated with the
skin, granular cell tumors
can cause a benign reaction
called pseudo-
epitheliomatous hyperplasia
of the overlying skin that
can mimic squamous cell
carcinoma, both grossly and
histologically

Fisher KW, Hattab EM. Nerve tumors. in Nerves and nerve injuries, vol 2. Pain, treatment, injury, disease, and future directions. Tubbs RS, Rizk E,
Shoja MM, Loukas M, Barbaro N, Spinner RJ (eds). Elsevier 2015
 RECURRENCE ?
• Curative treatment consists of local excision with
negative margins, resulting in a local recurrence
rate of less than 10%

• There is a tendency for recurrence of incompletely

resected tumors (5 out of 24 incomplete excision
(Lack)
Lack EE, Worsham GF, Callihan MD, Crawford BE, Klappenbach S, Rowden G, et al. Granular cell tumor: a clinicopathologic study of 110
patients. J Surg Oncol 1980;13:301–316.
 GRANULAR CELL NERVE TUMOR
OF THE PERIPHERAL NERVES
• Unfrequent (although of neural origin)

• 110 cases (95 pts) from Memorial Hospital over a 3I-year period. 7 involved the
fingers , two the palms, two the wrist and seven the arm (Strong).

• 110 patients from a 32-year period at the National naval medical center with no
nerve reported (Lack)

• In a 30-year review of 543 peripheral nerve tumors, only two GCTs were reported,
both of which involved the brachial plexus and not the peripheral nerve trunks (Kim).

• Ulnar nerve most frequently involve for unknown reason

Strong EW, McDivitt RW, Brasfield RD. Granular cell myoblastoma. Cancer 1970;25:415-21.
Lack EE, Worsham GF, Callihan MD, Crawford BE, Klappenbach S, Rowden G, et al. Granular cell tumor: a clinicopathologic study of 110
patients. J Surg Oncol 1980;13:301–316.
Kim DH, Murovic JA, Tiel RL, Moes G, Kline DG: A series of 146 peripheral non-neural sheath nerve tumors: 30-year experience at Louisiana
State University Health Sciences Center. J Neurosurg 102:256–266, 2005
Kim DH, Murovic JA, Tiel RL, Moes G, Kline DG: A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State
University Health Sciences Center. J Neurosurg 102:246–255, 2005
• Arai T, Furuya K, Kawaguchi N, Tanabe K, Amino
K (1977). Granular cell tumor of the peripheral
nerve and its histogenesis (author’s transl).
Seikeigeka, 28: 1061–1069 (in Japanese).

• (54 year-old woman who underwent complete

resection).
 AXILLARY NERVE
• 54 year-old woman

• Previous surgery for breast cancer

• Severe right arm pain with no weaknes

but diminish sensation in the axillary
nerve territory

• MRI revealed a 2,6x1,8x1,3 cm soft

tissue mass between long head of the
triceps and deltoid muscle

• Extirpation was possible from the

posterior branch of the axillary nerve

• Good results at 14 months FU

Mindea, SA, Kaplan, KJ, Howard, MA, O’Leary, ST. Granular cell tumor involving the axillary nerve: an unusual occurrence. Case report.
Neurosurg Focus. 2007, 22: E24,1-3
 RADIAL NERVE

• Nerve resection and graft was performed

Kato R, Kino Y, Hattori Y, et al. (1987). Granular cell tumor of the radial nerve in the upper arm. Seikeigeka, 38: 1589–1591.
PALMAR CUTANEOUS BRANCH OF THE MEDIAN 72 Condit and Pochron

NERVE

• 25 year-old female
Vol. 16A. No.1
January 1991 Granula

• 12 month history of a
painful volar 5 x 4 mm mass Fig. 1. Fusiform mass in the palmar cutaneous branch of the median nerve, radial to the palm
longus tendon.

right wrist, radial to palmaris

longus

• Pain was relief by surgery

(excision)
Fig. 3. Anesthetic area in the thenar region. 9 months after operation.

Condit DP, Pochron MD. Granular cell tumor of the palmar cutaneous branch of the median nerve. J Hand Surg 1991;16A:71–75
could not be dissected free from the ner ve. The surrounding tumors . This protein is found only in n
soft tissue was not involved. The mass and 2 to 3 mm of The majority of the present literature s
nerve, proximally and distally, were excised. cept of a neural origin for this tumor. \
 DIGITAL CUTANEOUS NERVE

• Weinreib et al. biopsied one of a patient’s

numerous small digital nodules; it was consistent
with a granular cell tumor arising from a cutaneous
sensory nerve.

Weinreb I, Bray P, Ghazarian D. Plexiform intraneural granular cell tumour of a digital cutaneous sensory nerve. J Clin Pathol 2007;60: 725–726.
DIGITAL NERVE: 4 REPORTED CASES

• Slutsky DJ. Granular Cell Nerve Tumor in the Hand: Case Report. J
Hand Surg 2009;34A:1512–1514.

• Enghardt MH, Jordan SE. Granular cell tumor of a digital nerve.

Cancer 1991;68:1764–1769.

• Bue P, Holck S, Holst-Nielsen F. Granular cell tumor localized in a

digital nerve [in Danish]. Ugeskr Laeger 1984;146:2319–2320.

• Ha SY, Suh YL, Sung CO. Granular cell tumour arising in a digital
nerve. J Hand Surg Eur Vol. 2011 Mar;36(3):249-50.
 DIGITAL NERVE GRANULAR CELL NERVE TUM

• 12 years old girl

• 5-year history of an isolated,

tender nodule along the volar
aspect of her left index finger that
1513
was exacerbated by percussion
GRANULAR CELL NERVE TUMOR IN THE HAND

FIGURE 1: There was a lobular yellowish lesion (arrow) FI

and gripping. attached to the ulnar digital nerve proper. fas
gr

• 2-cm interpositional graft was or

se
performed Sh
ne
ne
• No recurrence at 1 year al
pr
• Slight
FIGUREdiminished
1: There was a sensibility
lobular yellowish lesion (arrow) FIGURE 3: Second surgery, with excision of the remaining m
na
attached to the ulnar digital nerve proper. fascicle to achieve wide margins and interposition of a 3-cm
Slutsky DJ. Granular Cell Nerve Tumor in the Hand: Casegraft harvested
Report. from
J Hand the2009;34A:1512–1514.
Surg lateral antebrachial cutaneous nerve. te
 PATHOLOGY
F
• An expanded, cord-like structure FIGURE 1: There was a lobular yellowish lesion (arrow)
attached to the ulnar digital nerve proper. f
containing cells with small bland g

nuclei and abundant granular o

eosinophilic cytoplasm extending to s
S
the surgical mar- gins. There was no n
evidence of substantial mitosis, n
cellular atypia, or necrosis. The a
p
lesion was entirely composed of m
granular cells, with no myxoid n
t
stroma or wavy neurofibroma type
cells. The cells stained positively D
G
with a periodic acid–Schiff stain, The FIGURE 2: Hematoxylin-eosin stain of an encapsulated lobular b
granular cells were positive for mass with a fibrovascular connective tissue perineurial coat T
(magnification 25!).
S-100 and negative for cytokeratin. s
r
The granular cells were positive for S-100 and neg- n
Slutsky DJ. Granular Cell Nerve Tumor in the Hand: Case Report. J Hand Surg 2009;34A:1512–1514.
ative for cytokeratin. The S-100 is an acidic protein that m
 DIGITAL NERVE

• 23 year-old male

• A firm subcutaneous nodule exquisitely tender to palpation

at the distal flexion crease.

• The ulnar digital nerve had a fusiform nodular mass 2 mm in

diameter and 8 mm in length.

• No recurrence but no FU given

Enghardt MH, Jordan SE. Granular cell tumor of a digital nerve. Cancer 1991;68:1764–1769.
 DIGITAL NERVE

• No details available

Bue P, Holck S, Holst-Nielsen F. Granular cell tumor localized in a digital nerve [in Danish]. Ugeskr Laeger 1984;146:2319–2320.
 DIGITAL NERVE
• 18 year-old woman

• 2 years history of tingling

sensation left small finger

• Sonogrpahy revealed a small

nodule arising from the
collateral nerve, 2x1,5x1,5
mm.

• The lesion was dissected free

from the nerve.
Ha SY, Suh YL, Sung CO. Granular cell tumour arising in a digital nerve. J Hand Surg Eur Vol. 2011 Mar;36(3):249-50.
 ULNAR NERVE
• Davis GA. Granular cell tumor: a rare tumor of the ulnar nerve. Case
report. Neurosurg Focus 2007;22:1–5.

• Dahlin LB, Lorentzen M, Besjakov J, Lundborg G. Granular cell tumour of the

ulnar nerve in a young adult. Scand J Plast Reconstr Surg Hand Surg
2002;36:46–49.

• Yasutomi T, Koike H, Nakatsuchi Y. Granular cell tumour of the ulnar nerve. J

Hand Surg 1999;24B:122–124.

• Whadwa V, Salaria SN, Chhabra A. Granular Cell Tumor of the Ulnar Nerve:
MR Neurography Characterization. Radiology Case. 2014 Jun; 8(6):11-17
 ULNAR NERVE
• All had some combination of numbness, weakness, wasting, and positive Tinel’s sign.

• In the case reported by Yasutomi et al., the tumor was shelled out. At 2 years, the pain had resolved, but
the patient had persistent weakness.

• In the case reported by Dahlin et al., the nerve was so intimately involved that the authors were unable
to resect the tumor and performed a biopsy only. Because of increasing weakness and enlargement of
the tumor size to 4 cm, another biopsy was performed 2.5 years later. There was no change in the
histologic findings, and the authors continued to observe the tumor.

• In the case reported by Davis, the patient had muscle grade of 0 power (no contraction) of the
interossei and abductor digiti minimi, with grade 2 power (antigravity) in the ulnar innervated lumbricals.
Davis performed an interfascicular neurolysis and resected a 4.5-cm tumor, preserving 3 normal fascicles.
The defect was reconstructed with a 5-cm nerve graft harvested from the medial antebrachial
cutaneous nerve. At 12 months, the patient reported improved sensory function and had regained
grade 3/5 power (against mild resistance) of abduction but no adduction of the fingers.

• In the case of Whadwa, resection of the tumor while preserving the motor branch was possible.
 ULNAR NERVE
• 51-year-old, right-handed man with a 3-month history of numbness on the
ulnar side of the right hand and a 2-month history of weakness of thumb
and little finger adduction

• A tender 1.5 × 1.0 cm mass 3 cm proximal to the distal wrist crease on the
ulnar side of the palmaris longus tendon.

• Atrophy of the hypothenar eminence and dorsal interossei, and manual

muscle testing demonstrated weakness in all the interossei and the
abductor digiti minimi. Froment’s sign was positive, but there was no clawing
of the ring or little fingers. He became unable to use chopsticks due to
weak adduction of the thumb

Yasutomi T, Koike H, Nakatsuchi Y. Granular cell tumour of the ulnar nerve. J Hand Surg 1999;24B:122–124.
 We present a rare case of a granular cell tumour arising
Granular cell tumour is a relatively common and usually
in the ulnar nerve. longitudinally. The tumour compressed and displaced
benign neoplasm that most often occurs in the tongue, the fascicles of the ulnar nerve towards the ulnar side.
skin and subcutaneous tissue. The histogenesis of this Macroscopically, no nerve fascicles were involved and
CASE REPORT tumour is controversial. Many reports advocate the the tumour was dissected from around the fascicles and
concept that this tumour is of Schwann cell origin shelled out. The excised tumour measured 1.5 × 0.7 × 0.7
A 51-year-old, (Fisher
ULNAR NERVE
and Wechsler,
right-handed man 1962; Stefansson with
presented and Wollmann,
a cm (Fig 2). It was encapsulated by smooth fibrous tissue
1982). Nevertheless, a granular cell tumour arising in a and revealed a solid, homogeneous, light yellow cut
3-month historyperipheral
of numbnessnerveon the is
trunk ulnar side of the
an extremely rareright
occurrence. surface.
hand and a 2-month history
We present ofcase
a rare weakness of thumb
of a granular and arising
cell tumour
little finger in
adduction. Except for non-insulin-
the ulnar nerve.
dependent diabetes mellitus diagnosed 10 years previously,
his past and family
CASE history
REPORT disclosed nothing remarkable.
• A Physical
firm, examination oftumour
vermiform the right forearm
involving revealed a
A 51-year-old, right-handed man presented with a
tender 1.5 × 1.0 cm mass
3-month history3 of
cmnumbness
proximal on thetoulnar
the side
distal
of the right
the
wrist ulnar nerve
crease on the and
hand located
ulnar inside
side ofhistory
a 2-month the palmaristhe longus
of weakness of thumb and
tendon. The mass littlewasfinger
firm and rubberyExcept
adduction. and it for was non-insulin-
not
epineurium without
fixed to the skindependent
or the underlying adhesion
diabetes mellitus
tissue. to
diagnosed
Percussion10 years previously,
over
surrounding
the mass elicited soft
his past
tissues.
and family history
paraesthesia in the disclosed
ringnothing remarkable.
and little
Physical examination of the right forearm revealed a
fingers. The sensations
tender 1.5of× light 1.0 cmtouch
mass and 3 cmpinprick
proximal were to the distal
Fig 1 Intraoperative photograph. Digits are to the right and the
decreased in these fingers and over the right palm.
wrist crease on the ulnar side of the palmaris longus There elbow to the left. The lesion is seen on the radial side of the
• Macroscopically,
was atrophy of the
tendon.
fixed to
noskinnerve
hypothenar
The
the
mass
or
was
the
fascicles
eminence
firm and
underlying
and
rubbery
tissue.
dorsal
and it
Percussion
was not
over
ulnar nerve.
interossei, and manual muscle testing demonstrated
were
weakness involved and
in allthethemassinterosseithe paraesthesia
elicited tumour
and was
in
the abductor the ring and little
digiti
fingers. The sensations of light touch and pinprick were
dissected
minimi. from
Froment’s sign
decreased
clawing of the ring
around
inwas
these the
positive,
fingers andfascicles
but there
over waspalm.
the right no There Fig 1 Intraoperative photograph. Digits are to the right and the
elbow to the left. The lesion is seen on the radial side of the
was or little of
atrophy fingers.
the hypothenar eminence and dorsal
and shelled interossei,
Radiographs out
of the and wrist
manual weremuscle normal.testing Ultra-
demonstrated
ulnar nerve.

sonography using weakness

a 10-MHz in all the interossei
scanner and thea abductor
showed hypo- digiti
echoic mass thatminimi.
measured Froment’s
1.7 ×sign
0.8 was
× 0.7 positive,
cm, inbut there was no
contact
• Two
with theyears after surgery, there was
clawing of the ring or little fingers.
ulnar nerve.Radiographs of the wrist were normal. Ultra-
Over the following few weeks,
using a the feeling of numbness
no recurrence
in the right hand
sonography
echoic
or
gave
metastasis,
massway that to
10-MHz
a prickly
measured
but
scanner
1.7 × pain,
he
0.8 × 0.7
showed a hypo-
andcm, he
in contact
still
becamecomplained with
unable to use
Over
of
the
slight
thechopsticks
ulnar nerve. due
following
hypaesthesia
few
to weak adduction
weeks, the feeling
of the thumb. No apparent change of the mass ofwas numbness
in the little
detected finger
in the right
by palpation. A andhand mild
gave way
neurilemmoma
became unable to use chopsticks
weakness
to a prickly pain, and he
wasduesuspected,
to weak adduction
of the interossei
and the mass was surgically removed.
of the thumb. No apparent change of the mass was
Exploration detected
revealed a firm, Avermiform
by palpation. neurilemmomatumour was suspected,
and the
involving the ulnar mass(Fig.
nerve was surgically
1). It was removed.
located inside
Yasutomi T, Koike
Exploration H, Nakatsuchi
revealed
the epineurium without adhesion to surrounding Y.
a Granular
firm, cell tumour
vermiform of the ulnar nerve. J Hand Surg 1999;24B:122–124.
softtumour
involving the ulnar nerve (Fig. 1). It was located inside
 ULNAR NERVE
• 36 year-old right-handed woman

• 1 year history of pain in the

hand, mostly medial side

• Some wasting of the interossei

and diminished sensation in the
ulnar nerve territory (except for
FCU and FDP)

• Sonography reveals a swelling of

the ulnar nerve proximal to the
wrist.
Davis GA. Granular cell tumor: a rare tumor of the ulnar nerve. Case report. Neurosurg Focus 2007;22:1–5.
• The 4.5-cm tumor was identified
within the nerve, and then an
interfascicular neurolysis was
performed. This procedure
allowed three normal fas- cicles
to be dissected off the tumor
and preserved. All the remaining
ulnar nerve fascicles were
intimately involved in the tumor
itself and could not be dissected
off it. There- fore, an en bloc
resection was performed, and
the involved nerve fascicles
were resected together with the
tumor. The resulting defect was
repaired with a 5-cm nerve graft
har- vested from the medial
cutaneous nerve of the forearm.
Davis GA. Granular cell tumor: a rare tumor of the ulnar nerve. Case report.
Neurosurg Focus 2007;22:1–5.
 ULNAR NERVE
• 32 year-old woman

• Gradual weakness as well as inability

to use her right fingers during fine
manipulation.

• Progressive atrophy 1st dorsal web

• Positive Froment’s sign

• MRI found a tumor distal to the Guyon’s

canal

• Mass was dissected from within the ulnar

nerve with careful preservation of motor
fascicles. FU was only of 3 months
Whadwa V, Salaria SN, Chhabra A. Granular Cell Tumor of the Ulnar Nerve: MR Neurography Characterization. Radiology Case. 2014 Jun;
8(6):11-17
 SUPRASCAPULAR NERVE
• Malignant

• 48 year-old orthopedist with shoulder

motion limitation > 10 months

• Mass 22x13x6 cm

• No clinical aggravation

• The immunohistochemical staining

studies of MGCT revealed, S-100
protein, and NSE were strongly positive
in almost all MGCTs. CD68 was positive
in part of the cases.

• No recurrence at 1 year
Jia W, Chen C, Chen L, Yu C, Kondo T. Large malignant granular cell tumor with suprascapular nerve and brachial plexus invasion. case report
and literature review. Medicine (2017) 96:44:1-3
 OUR EXPERIENCE

• 4 x 3 mm soft tumor arising from the radial dorsal

collateral nerve of the ring finger, right hand

• Complete resection

• No recurrence known, no pictures available

 OUR EXPERIENCE
• 31 years-old black female, right handed

• Progressive aggravation of dysesthesiae in the left ulnar

nerve territory

• Palpation of a painful soft tumor 5 cm above the medial

epicondyle

• Weakness of interossei (20 vs 50 £)

• Sonography revealed a 3 cm long, intra-neural tumor

• MRI showed an intra-neural lesion

• 1st surgery 08/2017: intra-neural tumor that was not

removable

• Persistent hyperesthesiae, and progressive worsening

• Sonography showed an increase of the tumor

• Planned for complete excision + graft + IOA nerve

transfer over the ulnar motor branch at the wrist