Abstract

Glomus tumors are rare tumors primarily found on the nail bed of the fingers and rarely occur in other parts of the body. We present a case of a 71-year-old male who came in with a 5-year history of glomus tumor (GT) of the right lateral thigh associated with progressively increasing intermittent sharp pain. He denied any numbness or paresthesias of the extremity. Physical examination revealed a tender mass on the lateral side of the right upper leg associated with edema and erythema. Surgical excision and immunohistochemistry were performed. A histopathological diagnosis of glomus tumor was made. The postoperative recovery was successful and the patient reported resolution of the pain.

We performed a literature review of reported cases of GTs of the thigh. The review showed that all patients had a long period of symptomatic disease before an accurate diagnosis could be made and were easily treated with surgical excision. No evidence of recurrence was noted after surgical removal. GTs of the thigh rarely recur due to their solid and encapsulated structure. This also makes them easy to recognize and surgically remove.

This study highlights the unusual location of the GT, its impact on our patient, and reviews relevant academic literature. Tumors in these areas are frequently misdiagnosed due to vague symptoms. GTs should be included in the differential diagnosis of patients who experience pain with nonspecific symptoms that are not limited to specific body locations

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Introduction

Glomus tumors(GT) are benign mesenchymal neoplasms of vessels and glomocytes. They typically present as a bluish, painful lesion primarily found on the nail bed of the fingers and rarely occur in other parts of the body [1]. An unusual location often leads to missed or delayed diagnosis and management. In this study, we present a new case of GT located in the right lateral thigh of a 71-year-old male associated with intermittent sharp pain in his right thigh for 5 years. The pain resolved completely after the resection surgery. We study the unusual location of the GT, its effects on our patient, and review the academic literature pertaining to the topic.

Case report

A 71-year-old male presented with a history of painful mass in his right thigh for 5 years. The mass did not change in size and was associated with sharp intermittent pain radiating to the knee. The pain had been progressively increasing in intensity for the past 1-2 years and had acutely worsened in the last 3 months. It was characterized as 9/10 in intensity, persistent at night, and worse with walking or activity. The patient did not report any numbness or tingling of the extremity.

Physical examination revealed a tender mass on the lateral side of the right upper leg associated with edema and erythema.

Figure 1. Magnetic Resonance Imaging (MRI) revealed a 11x11x12 mm (AP x ML x CC) subcutaneous soft tissue mass peripheral to the caudal margin of the right greater trochanter. (A) and (B): Iso-Intensity on Coronal, Axial T1 weighted images with a vascular feeder (B, Yellow Arrow) (C): Coronal STIR MRI showing a mass(Red Arrow) with MRI marker(Yellow Arrow) (D): Uniform hyperintensity on fat suppressed T2 weighted images.

Magnetic Resonance Imaging (MRI), revealed a 11x11x12 mm (AP x ML x CC) subcutaneous soft tissue mass peripheral to the caudal margin of the right greater trochanter with a vascular feed (Fig. 1A and B, yellow arrow). Coronal STIR MRI showed a mass (Fig. 1C, red arrow) with MRI marker (Fig. 1C, yellow arrow). Isointensity on T1 weighted images, and uniform hyperintensity on fat suppressed T2 weighted images were also noted (Fig. 1D). The lesion was located within 3mm of the skin surface.

Surgical excision and immunohistochemistry was performed. The tumor was well-circumscribed with a thin capsule-shaped rim of fibrous tissue, surrounded by native adipose tissue (Fig. 2A). The bland round cells typical of a glomus tumor were observed at high power magnification (Fig.

2B). Blood vessels were seen throughout (Fig. 2B, yellow arrows) and were quite prominent – a finding seen in overtly vascular glomus tumors, sometimes called glomangiomas. Nerves are also present and may partially explain the painful nature of these tumors (Fig. 2B, blue arrow). The mass was SMA + (smooth muscle actin) (Fig. 2C), calponin + (highlights myoepithelial cells), CD34 + (faint blush) (Fig. 2D), CD31 + (highlights blood vessels), and S100 + (highlights nerves) while negative for synaptophysin, chromogranin, and pancytokeratin. Ki-67 was <1%. A histopathological diagnosis of glomus tumor was made.

The postoperative recovery was successful. The patient had no complaints regarding his surgical excision and the pain subsided immediately.

Figure 2. (A)Low power magnification reveals a well-circumscribed tumor with a thin capsule-like rim of fibrous tissue, surrounded by native adipose tissue. (B)High power magnification reveals bland, round cells, typical of a glomus tumor. Blood vessels are seen throughout(yellow arrows) and are quite prominent—a finding seen in overtly vascular glomus tumors, sometimes called glomangiomas. Nerves are also present and may partially explain the painful nature of these tumors. One nerve can be seen in the middle of this image (blue arrow). (C) Immunohistochemical staining for smooth muscle actin is positive (brown) in glomus tumors/glomangiomas. (D) Immunohistochemical staining for CD34 highlights the vascular component.

Table:1

Cases/year Age Gender Localisation Size Surgery Followup
1* 71 M Right thigh 1.1 x 1.1 x 1.2 cm on MRI excision symptom-free for 5 months of follow-up
2/2009 Hakverdi, Sibel et al. (6) - - left hip 1.2 cm (ultrasound) excision symptom-free for 3 months of follow-up
3/2003 Gencosmanoglu, Rasim et al. (7) 68 Male Right hip 2 cm (ultrasound) excision symptom-free for 2 months of follow-up
4/2008 Weiser, Jessica A et al. (8) 57 Male Left lateral hip 3.1x2.2x1.5 cm (on histopathologic examination) excision Fully recovered at discharge
5/2023 Pena-Burgos, E M et al. (9) 79 Male Thigh location 9.5cm excision No local relapse
6/1997 Amillo S, Arriola FJ et al. (10) 38 Female Thigh 3cm (MRI) Excision symptom-free for 12 months of follow-up
7/1989 Inoue O, Ibaraki K et al. (11) 26 Female Gluteal region 5x3x1.5cm excision No recurrence at 1.5 years after surgery
8/2005 Hermann, G., Klein, M.J., Springfield, D. et al. (12) 36 Female Mid-thigh 0.9x2cm (MRI) excision symptom-free for 12 months of follow-up
9/2014 Ezeoke C, Xiang D et al. (13) 48 Female Right thigh 3.1x2.2cm (MRI) - Excision with re-excision in 5 months (due to the positive margin of the initial biopsy for tumor cells) Disease free with no clinical evidence of recurrence
10/2014 So, Sang Young et al. (14) 65 Male Left anterior thigh 0.8x0.6 cm2 hypoechoic cyst excision Pain free for six months follow-up
11/2017 Margad, Omar, and Nabile Bousselmame. (15) 40 - Right thigh 1.5 cm excision Disease free for 2 years of follow-up
12/2015 Beksaç K, Dogan L et al. (16) 39 Male Posterolateral side on left thigh 15x10mm (CT) excision Pain free after surgery
13/2016 Lee TS, Wu HT, Chan RC et al. (17) 66 Female Lateral side of the right thigh 5mm Excision Symptom-free after surgery
14/2018 Sbai, Mohamed Ali et al. (18) 25 Female Posterior side of the left thigh 3x2x2 cm (MRI) excision Pain-free after surgery for the last year
15/2022 Avery, Emma Claire et al. (19) Mid-50s Female Right thigh - excision Symptom-free after surgery
16/2013 Dabadie, Alexia et al. (20) 13 Female Right thigh 3x5 cm highly calcified tumor excision No recurrence after 2 years of follow-up
17/2023 Alfattni, Ammar Abdulqader et al. (21) 39 Male Left thigh 9x9 mm (MRI) Incision Symptom-free after surgery
18/2012 Werner, Jeff D et al . (22) 48 Male Left thigh 6x6x4 mm (ultrasound) excision -
19/1997 Negri, G et al. (23) 21 Female Left thigh 22x11x6 cm of surgical specimen resection Symptom-free after surgery
20/2008 Sraj SA, Khoury NJ et al. (24) 48 Male Distal thigh (over the adductor canal) 1x1 cm excision Symptoms-free after surgery
21/2020 Kloping, Laskar Pradnyan et al. (25) 56 Male Posteromedial of the distal femur 6 x 5.3mm excision No recurrence after 6 months of follow-up
22/2012 Lancerotto, Luca et al. (26) 41 Male Lateral aspect of the intermediate third of right leg 2cm nodule Surgically excised Disease free for 3 years of follow-up
Table 1.

Dıscussıon

GTs are benign mesenchymal neoplasms composed of various proportions of vessels and glomocytes [1], typically present with hypersensitivity to cold, paroxysmal pain and point tenderness [2].They account for less than 2% of soft tissue cancers. The cells that make up the glomus body resemble modified smooth muscle cells. They are uncommon and sometimes misinterpreted as hemangiomas or venous malformations. Glomus tumors are categorized into three types [3] based on their dominant component:Solid; dominantly glomus cells, glomangioma; dominantly blood vessels and glomangiomyoma; dominantly smooth muscle cells.

According to the literature, GTs can be inherited or sporadic. The inherited type is autosomal dominant with incomplete penetrance and variable expression. The sporadic type has de-novo mutation and is present during birth. They are classically red, purple, blue or even skin-colored, which makes it even more challenging to diagnose. They are found in nail fold, hand, lower limb, middle ear, jugular bulb, and carotid artery. A differential diagnosis of the tumor includes a fibroma, hemangioma, leiomyoma, blue nevi, venous malformation, subungual exostosis, and mucoid cyst [3].

GTs clinically diagnosed, and preferred methods for diagnosing GTs are MRI and ultrasound. Ultrasonography, especially color-duplex ultrasonography can be useful in detecting small nodules that appears hypoechoic or isoechoic nodules with hypervascularity on Doppler [4-5].

Immunohistochemistry can further assist in diagnosis as they are a-smooth muscle actin (aSMA), muscle-specific actin (MSA), h-caldesmon positive, and have abundant type IV collagen [1-3].

The treatment of choice is wide local excision [6] followed by monitoring for local recurrence. Reported recurrence rate is approximately 10%[1].

Glomus tumors constitute less than 2.0% of all primary soft tissue tumors, approximately 80% of the lesions are located in the upper extremity, and over 75% are subungually located [1]. Ultrasound and MRI are used for assessment of the lesions. A complete excisional biopsy is helpful in making a definitive diagnosis.

Glomus tumors constitute less than 2.0% of all primary soft tissue tumors, approximately 80% of the lesions are located in the upper extremity, and over 75% are subungually located [1].Ultrasound and MRI are used for assessment of the lesions. A complete excisional biopsy is helpful in making a definitive diagnosis.

We performed a literature review of reported cases, to the best of our knowledge, we have found 67 published cases in lower extremity rare area glomus tumors which is mentioned briefly in Table 2.

In this study, we mostly focused on rarer area, hip and thigh glomus tumors. We reviewed 21 cases of thigh glomangiomas from 1989 to 2023. Previously reported glomus tumors in the thigh/hip area including our study: cases, age, sex, location, size, surgery, follow up are shown in Table 1.

Tumor developed in 11 men, 9 women, gender of 2 patients was not documented. Patient age ranged from 13 to 79 (median age:48) The size of the tumors varies from 0.6 cm to 9.5cm. One tumor was excised with surgical specimen, measured 22 x 11 x 6 cm, consisting in parts of musculus vastus medialis and intermedius due to infiltrative growth. One tumor was described as a round hypoechoic cyst on ultrasonography. Ultrasound and MRI were used for assessment of the lesions. A complete excisional biopsy helps to make a definitive diagnosis.

Upon reviewing the cases we can summarize that all patients had a long period of symptomatic disease before making an accurate diagnosis from 1 month to several years. No malignancies were reported in these studies. All glomangiomas were treated with surgical excision with no evidence of recurrence. Glomus tumors in the thigh region rarely recur due to their solid and encapsulated structure, which makes them easy to recognize and surgically remove.

Conclusions

Commonly, Glomus tumors in these areas are misdiagnosed due to vague symptoms such as radiating pain and absence of sensitivity to cold and tenderness. Without a palpable mass, pain in this area may be masked as neuropathic; thus, Glomus tumor should be included in the differential diagnosis of patients who describe pain with vague symptoms without being restricted to certain body regions.

Declarations:

Ethical Approval: We affirm that the submitted work complies with all applicable ethical guidelines and regulations, including but not limited to the Declaration of Helsinki and the International Committee of Medical Journal Editors (ICMJE) guidelines.Informed Consent and Consent to Publish Identifiable Information or Images was obtained. Privacy and confidentiality were strictly maintained.

Competing Interests: The authors declare that they have no competing interests of a financial or personal nature that could potentially influence the results or interpretation of this study.

Authors' Contributions: The contributions of each author are as follows: Corresponding Author; Suheyla Karaduman wrote the main manuscript, Sarvinoz Albalushi, Alesia Talpeka and Suheyla Karaduman reviewed literature and prepared tables. Daniel Levitan prepared pathology figures. Nishhta Nigam wrote abstract. All authors have read and approved the final version of the manuscript.

Funding: This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Availability of Data and Materials: The datasets analyzed during the current study are available from the corresponding author upon reasonable request.

References

  1. Gombos Z, Zhang PJ: Glomus tumor. Arch Pathol Lab Med. 2008, 9:1448-52. 10.5858/2008-132-1448-GT
  2. McDermott EM, Weiss AP: Glomus tumors. J Hand Surg Am. 2006, 31:1397-400. 10.1016/j.jhsa.2006.05.018
  3. Liapi-Avgeri, G.; Karabela-Bouropoulou, V.; Agnanti, N. (1994: Glomus tumor: A histological, histochemical and immunohistochemical study of the various types. 190:2-10. 10.1016/S0344-0338(11)80490-5
  4. Glazebrook KN, Laundre BJ, Schiefer TK, Inwards CY: Imaging features of glomus tumors. Skeletal Radiol. 2011, 40:855-62. 10.1007/s00256-010-1067-1
  5. Stacy GS, Bonham J, Chang A, Thomas S: Soft-Tissue Tumors of the Hand-Imaging Features. Can Assoc Radiol J. 2020, 71:161-173. 10.1177/0846537119888356
  6. Hakverdi S, Kalaci A, Yaldiz M, Akansu B: Glomus tumor of the hip. An unusual location. Saudi Med J. 2009, 30:299-301.
  7. Rasim Gencosmanoglu, Resit Inceoglu, Ozlem Kurtkaya-Yapicier: Glomangioma of the hip. Dermatol Surg.2003 Dec. 29:1244-7. 10.1097/SCS.0b013e3181bec705
  8. Jessica A Weiser, Kristin Magnuson Nord, Marc E Grossman: Glomus tumor masquerading for 22 years as osteoarthritis of the hip. 2008:339-42.
  9. S.Amillo, f. J.Arriola, G. Mun ̃oz. Extradigital glomus tumor causing thigh pain. J Bone Joint Surg [Br. 1997, 79:104-6. 10.1302/0301-620X.79B1.0790104
  10. Osamu Inoue, Kunio Ibaraki, Hiromichi Norimatsu, Satoshi Tomiyama, Shin Zamami, Yoshisada Shingaki and Takeshi Nakama: A Rare Case of Ectopic Glomus Tumor in Gluteal Muscle. School of Medicine, University of the Ryukyus. 1989, 38:752-755. 10.5035/nishiseisai.38.752
  11. E. M. Pena-Burgos, G. Serra-Del Carpio, M: Tapia-Viñe, E: J. Ortiz-Cruz, J. J. Pozo-Kreilinger. Giant thigh glomus tumor of uncertain malignant potential: Case report with pathologic-radiologic correlation. Journal of Cutaneous Pathology. 2023, 50:390-395. 10.1111/cup.14416
  12. Hermann, G., Klein, et al.: Glomus tumor of the thigh: Confluent with the periosteum of the femur. Skeletal Radiology. 2005, 34:116-20. 10.1007/s00256-004-0768-8
  13. Ezeoke C, Xiang D, Poddar N: A rare case of glomus tumor of the thigh: Malignant or not?. Int J Case Rep Images. 2014, 5:818-821. 10.5348/ijcri-2014140-CR-10451
  14. Sang Young So, Byng Mook Kim, Sun Yeul Lee, Young Kwon Ko: Yong Sup Shin: and Won Hyung Lee. Glomus Tumor Causing Anterior Thigh Pain: A Case Report. Korean J Pain. 2014, 27:174-177. 10.3344/kjp.2014.27.2.174
  15. Omar Margad, Nabile Bousselmame: Glomus tumor of the thigh: a new case report and literature review. Pan Afr Med J. 2017, 26:73. 10.11604/pamj.2017.28.73.12827
  16. Kemal Beksaç, Lutfi Dogan, Nazan Bozdogan, Gulay Dilek, Gokhan Giray Akgul, Cihangir Ozaslan: Extradigital Glomus Tumor of Thigh. Case Rep Surg. 2015, 638283:10.1155/2015/638283
  17. Tzu-Shiuan Lee, Hung-Ta Wu, Rai-Chi Chan, Jia-Chi Wang: Sonographic diagnosis of a glomus tumor of the thigh. J Clin Ultrasound. 10.1002/jcu.22381
  18. Mohamed Ali Sbai, Sofien Benzarti, Wafa Gharbi, and Riadh Maalla: A Rare Case of Glomus Tumor of the Thigh with Literature Review. J Orthop Case Rep. 2018, 8:22-24. 10.13107/jocr.2250-0685.1192
  19. Emma Claire Avery, Haley Augustine, Shachar Sade, Jana Dengler: Extradigital glomus tumour of the thigh causing 8 years of undiagnosed debilitating pain. BMJ Case Rep. 202219, 15:249869. 10.1136/bcr-2022-249869
  20. Alexia Dabadie, Carla Fernandez, Guillaume Gorincour, Michel Panuel, Philippe Petit: A rare case of a calcified glomus tumour in the thigh of an adolescent. Pediatr Radiol. 2013, 43:1045-8. 10.1007/s00247-013-2640-2
  21. Ammar Abdulqader Alfattni, Wael Hyderabad, Khalid Saleem Alharthi, Ali Hadi Alyami: Glomus Tumor Within the Tensor Fascia Lata: A Case Report. Am J Case Rep. 2023, 24:938726. 10.12659/AJCR.938726
  22. Jeff D Werner, Chadwick L Wright, O Hans Iwenofu, Sumukh B Patil, William T: C Yuh: Unusual motion detected on real-time sonography inside a glomus tumor in the thigh. J Clin Ultrasound. 2013, 41:183-6. 10.1002/jcu.21961
  23. G Negri, M Schulte: W Mohr: Glomus tumor with diffuse infiltration of the quadriceps muscle: a case report. Hum Pathol. 1997, 28:750-2. 10.1016/S0046-8177(97)90188-X
  24. Shafic A Sraj, Nabil J Khoury, Nadim E Afeiche, John Abdelnoor: Thigh pain of 5 years' duration in a 48-year-old man. Clin Orthop Relat Res. 2008, 466:2291-5. 10.1007/s11999-008-0136-4
  25. Laskar Pradnyan Kloping, Lukas Widhiyanto, Komang Agung Irianto, Oen Sindrawati: Yudhistira Pradnyan Kloping: Glomus tumor-induced lower extremity pain: A case report. International Journal of Surgery Case Reports. 2020, 75:352-356. 10.1016/j.ijscr.2020.09.093
  26. Lancerotto L, Salmaso R, Sartore L, Bassetto F: Malignant glomus tumor of the leg developed in the context of a superficial typical glomus tumor. Int J Surg Pathol. 2012, 20:420-4. 10.1177/1066896911432454