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Glomus tumor of the mesocolon

Luke Harpera,*, Frederic Lavranda, Brigitte Le Bailb, Muriel Brunc,Stephane Ferronc, Pierre Osesa, Pierre Vergnesa

aDepartment of Paediatric Surgery, Hopital Pellegrin-Enfants, CHU Bordeaux 33076, FrancebDepartment of Pathology, Hopital Pellegrin-Enfants, CHU Bordeaux 33076, FrancecDepartment of Radiology, Hopital Pellegrin-Enfants, CHU Bordeaux 33076, France

0022-3468/$ – see front matter D 2005

doi:10.1016/j.jpedsurg.2005.06.016

* Corresponding author. Tel.: +33 55

E-mail address: harper_luke@hotma

Index words:Glomus tumor;

Mesocolon;

Benign neoplasms

Abstract Glomus tumors are rare distinctive benign neoplasms, which arise from modified smooth

muscle cells of the normal glomus body and are most commonly located in the subungual region of the

finger. Intraabdominal locations are relatively rare. We report a case of glomus tumor of the mesocolon

in a 10-year-old girl. Surgical exploration showed a lesion in the transverse mesocolon, which was

excised. Histopathology showed it to be a glomus tumor of the mesocolon.

D 2005 Elsevier Inc. All rights reserved.

Glomus tumors are rare distinctive benign neoplasms,

which arise from modified smooth muscle cells of the

normal glomus body. They are most frequently located in

the subungual region of the finger and present as small blue-

red nodules in the deep dermis or subcutis. Other sites

include the palm, wrist, forearm, foot, and tip of the spine.

Rarely, they occur in the gastrointestinal tract (stomach,

small intestine, colon), trachea, lungs, bone, vagina, and the

cervix [1]. We report a case of glomus tumor of the

mesocolon in a 10-year-old girl.

1. Case report

A 10-year-old girl was transferred to our unit for

management of an intraabdominal mass. An echography

performed for uncharacteristic abdominal pain had revealed

a small, well-circumscribed hypoechogenous mass in front

of the left kidney. An abdominal scan had confirmed a

25-mm-diameter lesion seemingly in continuity with the tail

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il.com (L. Harper ).

of the pancreas (Fig. 1). At her arrival, the girl was

asymptomatic, and biologic tests including amylase, lipase,

cancer antigen 19-9, serum insulin, and C-peptide levels

were all normal. A magnetic resonance imaging scan was

then performed, both confirming the size and aspect of the

mass and further suggesting a pancreatic origin to the lesion.

Surgical exploration revealed a nodular tumor of the

Journal of Pediatric Surgery (2005) 40, E37–E38

Fig. 1 Injected abdominal scan showing the tumor in continuity

with the pancreas tail.

Fig. 2 Histologic aspect of the lesion.

L. Harper et al.E38

transverse mesocolon not connected to the pancreas. The

tumor was excised. Pathological analysis showed an

encapsulated heterogeneous nodule measuring 25 � 29 mm,

containing a monotonous population of small round cells

surrounded by myxoid stroma and large spaces of eosino-

philic necrosis (Fig. 2). Immunohistochemically, the cells

expressed muscular markers (a–smooth muscle actinin and

desmin) but were negative for endocrine tumor markers

CD34, c-kit, S-100 protein, anaplastic lymphoma kinase,

glial fibrillary acidic protein, a-fetoprotein, epitheleal

membrane antigen, myogenin, and caldesmon. MIB prolif-

eration index was estimated at 15%. Histologic diagnosis

showed it to be a glomus tumor of the mesocolon. The

postoperative course was uneventful, and on follow-up, the

girl is well.

2. Discussion

Glomus tumors are benign tumors, which arise from the

arterial portion of the glomus body or the Sucquet-Hoyer

canal, an arteriovenous shunt in the dermis that contributes

to temperature regulation. Although they arise from glomus

cells, these tumors have been observed in extracutaneous

locations that are not known to contain glomus cells—

trachea, stomach, small intestine, colon, lungs, bone, vagina,

and the cervix [2,3]. This could be explained by the fact that

these tumors may arise from perivascular cells that can

differentiate into glomus cells [1,4,5]. Multiple glomus

tumors, especially the disseminated variant, are inherited in

an autosomal dominant pattern with incomplete penetrance.

Although the initiating event for glomus cell proliferation is

unknown, some authors have postulated that trauma induces

solitary subungual glomus tumors [6]. A viral origin could,

however, be possible. In an experimental animal study

involving inoculation of an incomplete form of the influenza

virus, glomangioma of the mesentery was observed in

18.7% of mice [7]. Treatment is surgical excision, which, in

cases of multiple lesions, should be restricted to symptom-

atic lesions. The radiological aspect of this lesion was

compatible with the diagnosis of Frantz tumor. With

hindsight, on the magnetic resonance imaging scan, there

is a fine demarcation line between the tumor and the

pancreas on T2 weighted images. Morphologic and immu-

nohistochemical data are typical so distinction between

hemangiopericytoma, solitary fibrous tumor, or myoepithe-

lioma can be made. Histology confirmed the benign nature

of the lesion; however, malignant forms do exist. Gloman-

giosarcomas have more atypia, pleomorphism, and mitotic

figures, and they have an invasive growth pattern.

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