The first reports of glomus tumors date back to the
16
th century. Von Haller,[
2] in 1762, described a mass
at the carotid bifurcation area in a glomus body-like
structure. In 1812, Wood[
3] described a glomus tumor
as painful subcutaneous tubercles. In 1840, Valentin[
4]
defined it as ganglia tympanica. A glomus jugulare
tumor case was first reported by Rosenwasser[
5] in
1945. A long time after von Haller,[
2] Mulligan,[
6] in
1950, introduced the term chemodectoma to describe
glomus tumors for their chemoreceptor tissue origin.
In 1974, Glenner and Grimley[
7] renamed the tumor
as paraganglioma and introduced a classification
according to the localization, innervation, and
histopathological features of the tumor. Four types of
glomus tumors are defined according to its origin of
carotid body or nodosum of vagus: Type 1 is defined
as within 2 cm of carotid bifurcation and no cranial nerve deficits; Type 2 extending 2 cm beyond carotid
bifurcation or encases internal carotid artery; Type 3
extending within 2 cm of or through skull base; and
Type 4 bilateral/multiple and/or atypically located
tumors. In our case, the tumor was Type 4.
Rarely, as in our case, paraganglionic cells
of the extra-adrenal neuroendocrine system may
occur in various localizations of the body including
orbit oculi, pterygopalatine fossa, larynx, pharynx
and dermis, and upper and lower extremity.[8]
These cells in these localizations seem to involve
atypical accumulation of glomus caroticum cells.
Malignancy is related to size, deeper location,
infiltrative growth, mitotic activity, and nuclear
pleomorphism with necrosis.
The reported surgical mortality for carotid body
tumors of the neck is around 8 to 10%, which is
mainly due to a major neurological complication
and/or deficit at the postoperative period.[9] Glomus
tumors of the extremities are rarely reported in the
literature. Nonetheless, we believe that the mortality
may be significantly lower in these extremity regions
than the rate of neck tumors.
Alternative treatment techniques such as coil
embolization and gamma knife surgery have been
advocated in the literature.[10] Due to the dense
vascularity of these tumors, despite a preoperative
definitive diagnosis of these inner and surrounding
tumor vessels, a complete treatment by a coil
embolization and/or gamma knife surgery is difficult
to achieve. Furthermore, a massive bleeding during
these alternative approaches may complicate excision,
the second step of surgery, with higher morbidity
and mortality rates. In our opinion, a direct surgical
removal is the treatment of first choice, particularly in
cases larger than 2 cm.
In conclusion, glomus tumors of the lower extremity
are extremely rare. A total excision and removal of the
mass lesion can be achieved with appropriate measures.
Declaration of conflicting interests
The authors declared no conflicts of interest with respect
to the authorship and/or publication of this article.
Funding
The authors received no financial support for the research
and/or authorship of this article.