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CASE REPORT
Year : 2022 | Volume
: 26
| Issue : 3 | Page : 40-43
Glomus tumor presenting as atypical tender papule on the lower limb
Banupriya Mani, Anuradha Priyadarshini, Gayathri Rajesh, Anandan Sankarasubramanian
Department of Dermatology, Venereology and Leprology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
Correspondence Address:
Dr. Banupriya Mani
3/166, Sri Bhavani Illam, Anna Street, Madipakkam, Chennai - 600 091, Tamil Nadu
India
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/jdds.jdds_58_19
Glomus tumor is a benign vascular tumor derived from modified smooth muscle cells of the glomus body. Although they can develop in any part of the body, they commonly do so in the upper extremities, most frequently in the subungual region of fingers. They can be either solitary or multiple. Extradigital glomus tumors are often misdiagnosed owing to their nonspecific clinical presentations and unusual locations. We report two cases of extradigital glomus tumor occurring at unusual sites such as knee and thigh.
Keywords: Glomus tumor, knee, tender papule, thigh
Glomus tumors are rare, benign, vascular neoplasms constituting only 1.6% of all soft-tissue tumors.[1] They arise from the glomus body, a contractile neuromyoarterial structure found in the reticular dermis.[2] Their function is to adjust the blood pressure and temperature by directing blood flow within the subcutis.[3] They present most commonly in the subungual region of the distal phalanx, since they contain large number of the normal glomus body.[4] However, they are also reported in extradigital locations where clinical diagnosis can be made based on the classic triad of paroxysmal pain, tenderness, and cold hypersensitivity.[5] They are classified into solid glomus tumor, glomangioma, and glomangiomyoma based on the presence of variable quantities of glomus cells, blood vessels, and smooth muscle.[6]
Case ReportsA 31-year-old male came to the dermatology outpatient department with complaints of a single, painful, nonprogressive lesion below the left knee for a duration of 1 year. He had a history of exacerbation of pain on touch and exposure to cold. On examination, an erythematous, tender papule of size 0.25 cm × 0.25 cm was present inferior to the left knee. Surrounding skin was normal without any induration or extension [Figure 1]a and [Figure 1]b. A 42-year-old male presented with similar history on the right thigh for 6 months. On examination, a bluish, tender, compressible papule of size 0.5 cm × 0.5 cm was present on the anterolateral aspect of the right thigh [Figure 2]a and [Figure 2]b.
Figure 1: (a) An erythematous papule below the medial aspect of the left knee, (b) magnified view of the papule below the left knee
Figure 2: (a) A bluish compressible papule on the anterolateral aspect of the right thigh, (b) magnified view of the papule on the right thighIn both cases, differential diagnosis of eccrine spiradenoma, glomus tumor, blue rubber bleb nevi, and leiomyoma were considered. Excisional biopsy showed a well-circumscribed tumor in the dermis, composed of sheets and nests of monomorphic-rounded cells with eosinophilic cytoplasm and ovoid plump nuclei, surrounding capillaries [Figure 3]a and [Figure 3]b.
Figure 3: (a) Well-circumscribed tumor in the dermis with sheets and nests of monomorphic-rounded cells, surrounding capillaries H and E, ×10, (b) Dilated vascular spaces, surrounded by glomus cells (round cells with eosinophilic cytoplasm and ovoid plump nuclei) H and E, ×40In both cases, immunohistochemistry showed positive expression for smooth muscle actin (SMA), confirming the diagnosis of glomus tumor [Figure 4]a. CD34 was, however, negative [Figure 4]b.
Figure 4: (a) Positive expression for smooth muscle actin immunohistochemistry. (b) CD 34 negative. IHC: immunohistochemistry Discussion Glomus tumors, also known as Barré–Masson syndrome, were first described in 1924 by Masson.[2] They are benign hamartomas arising from the normal glomus apparatus in the subcutaneous tissue.
Glomus body is an arteriovenous anastomosis, and it functions as a receptor to regulate cutaneous blood pressure and temperature by modifying peripheral blood flow with their contractile ability.[1] Pain associated with glomus tumor is paroxysmal and disproportionate to size of the lesion, aggravated by tactile stimulus and exposure to cold.[4] Several hypotheses may explain the pathogenesis of pain. Cold temperature may trigger vasodilation of the glomus body resulting in pain, which is then exacerbated by pressure.[7] Other mechanisms include the release of substance P and contraction of myofilaments in glomus cells, resulting in an increase in intracapsular pressure which is then transmitted by the unmyelinated nerve fibers.[1] They also have abundant mast cells which release heparin, 5-hydroxytryptamin, and histamine causing receptors to react to pressure or cold stimulation.[2]
It is generally common in young adults between 20 and 40 years of age. The classical site of the subungual region is more common in females.[8] Extradigital sites are more common in males,[1] as seen in our cases. They may be solitary or multiple, appearing as pinkish, red or bluish, raised and painful nodule.[2] In both our cases, the lesions presented as papules as opposed to the usual nodules seen in this condition. The most common extradigital site is along the upper extremity, mostly the forearm, whereas the shoulder and upper back are least commonly involved.[9] Other extradigital cutaneous sites reported in the literature are lower limb [Table 1], chest, palm, and inguinal region.
Table 1: Summary of extradigital glomus tumors presenting as cutaneous lesions on the lower limb published in literatureDiagnosis is by excisional biopsy, which also happens to be the treatment of choice.[4] Histopathological analysis reveals a well-circumscribed proliferation of solid sheets and clusters of glomus cells surrounding vessels, as seen in our cases. The glomus cells are uniformly rounded or polygonal in shape and have centrally placed rounded nuclei and a pale eosinophilic cytoplasm.[8] Tumor cells are universally positive for SMA. However, positivity for CD34 is variable, which was negative in both our cases.[6]
Complete excision with meticulous care is curative for solitary lesions as the recurrence rate for incompletely excised tumors has been found to be between 12% and 33%.[7] Other modalities of treatment include electrodessication, sclerotherapy, CO2, and pulsed dye laser.[6]
In conclusion, glomus tumors are rare, extradigital glomus tumors even more so! We report the above cases of glomus tumor arising in the unusual sites of the knee and thigh, presenting as papules with the classical triad. Early detection, diagnosis, and treatment of this condition are of utmost importance to avoid chronic pain and restriction of the use of the affected limb. Complete surgical excision is mandatory to avoid recurrence and to be completely free from pain caused by residual tissue. This highlights that glomus tumors should be added to the list of the differential diagnosis for all painful dermal lesions with the classical triad of pain, cold sensitivity, and point tenderness.
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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
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