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Year : 2022  |  Volume : 65  |  Issue : 3  |  Page : 731-732
Primary glomus tumor of the liver: An uncertain malignant potential tumor

1 Department of Pathology, Shibei Hospital, Jing'an District, Shanghai, China
2 Department of Pathology, Jiangxi Maternal and Child Health Hospital, Nanchang, China
3 Department of Pathology, Fudan University Zhongshan Hospital, Shanghai, China
4 Department of Diagnosis, Ningbo Diagnostic Pathology Center, Ningbo, China

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Date of Submission26-Apr-2021
Date of Decision11-Aug-2021
Date of Acceptance22-Nov-2021
Date of Web Publication21-Jul-2022

How to cite this article:
Chang Y, He D, Ji Y, Ge R. Primary glomus tumor of the liver: An uncertain malignant potential tumor. Indian J Pathol Microbiol 2022;65:731-2

How to cite this URL:
Chang Y, He D, Ji Y, Ge R. Primary glomus tumor of the liver: An uncertain malignant potential tumor. Indian J Pathol Microbiol [serial online] 2022 [cited 2022 Aug 15];65:731-2. Available from: https://www.ijpmonline.org/text.asp?2022/65/3/731/351602

Dear Editor,

Glomus tumor (GT) is a stromal tumor originating from the neuromuscular vascular sphere surrounding the arteriovenous anastomosis. GT is composed of tumor cells with a resemblance of modified smooth muscle cells in the normal glomus body, most of which are present in the distal extremities.[1] GT seldom occurs in solid organs, since glomus bodies are absent or rarely present in these organs.[2] This report illustrates the presence of the glomus tumor in an unusual location.

A 59-year-old man presented with the right upper abdominal discomfort for over two months. Laboratory examinations show that he tested positive for HBsAg, HBeAg, and HBcAb. The liver function test showed ALT 24 IU/l, AST 23 IU/l, AIb 38 g/L, GIb 31.1 g/L, A/G 1.2, AFP 8.45 ug/L, and HBV-DNA 9.13 × 103 U/mL. The tumor markers (such as CEA-CA125-CA199-F-PSA-T-PSA-F-PSA/T-PSA) were within normal range. Ultrasonographic examination and CT scan showed a hepatic solid mass at the left lobe [Figure 1], which suggests the possibility of hepatic carcinoma. The patient underwent partial hepatectomy.

Grossly, partial liver tissue measured 5 cm × 4 cm × 4 cm. The cut surface revealed a well-demarcated tumor measuring 3 cm in diameter with a thin capsule, and a dark-red color and solid consistency [Figure 2]a Microscopically, the tumor cells were round or oval with uniform size and were arranged around the thin-walled blood vessels or sinuses in a hemangiopericytoma manner. Tumor nuclei were round with distinct nucleoli, no nuclear atypia, rare mitoses (0–2/50 HPF) without pathological mitoses [Figure 2]b. Immunostains showed that the tumor cells were diffused and strongly positive for SMA [Figure 2]c and calponin [Figure 2]d, but negative for Synaptophysin, Chromogranin A, CD56, Cytokeratin, Epithelial membrane antigen (EMA), S-100, HMB45, Desmin, and C-kit. The labeling index of Ki-67 (MIB-1) was <2%. The immunostain for CD34 highlighted the vascular network. Based on these findings, the patient was diagnosed with a primary liver GT. No further treatment was performed after the operation. Until now (72 months of follow-up), the patient has been well and symptom-free, with no evidence of tumor recurrence after the complete removal.
Figure 1: Computed tomography shows a hepatic mass at the left lobe

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Figure 2: (a) The tumor is dark-red in cut surface with focal hemorrhage. (b) Tumor cells are uniform in size with arrangement around the thin-wall blood (HE Stain × 100). (c) Tumor cells are positive for SMA (IHC Stain × 100). (d) Positive staining of calponin (IHC Stain × 100)

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sThe primary liver GT was first reported by Gassel et al.[3] Up to now, only 7 cases of liver GT have been reported in available studies except our case.[4],[5],[6] In contrast to ordinary lesions, which equally affect both sexes, liver GT may be prevalent in men. The mass diameter was 3–21 cm, which was substantially larger than common cutaneous GT. Five patients had symptoms of abdominal pain and discomfort, while the other two patients experienced the loss of appetite, weight loss and fullness in the epigastrium, and a long history of pain similar to cutaneous GT.[5] Liver GT may have different clinicopathological features from the soft tissues in some part.[6]

An analysis of 52 atypical and malignant GTs by Folpe et al.[1] proposed the following classification scheme. Malignant GT is defined as a tumor with a deep location and a size of more than 2.0 cm, or atypical mitotic figures, or moderate to high nuclear grade and more than 5 mitotic figures/50 HPF. Symplastic GT refers to a tumor with a high nuclear grade in the absence of any other malignant feature. A GT with an uncertain malignant potential is a tumor that lacks the criteria for malignant or symplastic GT but has a high mitotic activity and only a superficial location, or large size only, or with only a deep location. Our case showed a deep location and a size larger than 2.0 cm, but a low mitotic count without any histologic atypia or perineural or vascular invasion. Using this classification scheme, the tumor was discerned to be an uncertain malignant potential GT, hence, it is recommended that the patient should receive appropriate clinical follow-up.

The main differential diagnoses about this hepatic mass are as follows: (1) Angiosarcoma and epithelioid hemangioendothelioma. Generally, it is easy to be distinguished from angiosarcoma based on the lack of atypical cells and high mitotic figures. Furthermore, angiosarcoma and epithelioid hemangioendothelioma are positive for CD31 and negative for SMA, which is positive for GT. (2) Metastatic gastrointestinal stromal tumor (GIST). GIST is positive for CD117, CD34, and DOG-1, but often weakly positive or negative for SMA. (3) Perivascular epithelioid cell tumor (PEComa). The cells in the PEComa often surround thin wall vessels with transparent, granular, or light eosinophilic cytoplasm. Spindle-shaped smooth muscle cells are far from the vessels. All the tumor cells are positive for SMA, HMB45, and Melan-A. (4) Neuroendocrine tumor. This tumor is rich in blood sinus, while Keratin markers, Synaptophysin, Chromogranin A, and CD56 are typically positive, but negative for SMA.

In summary, the primary liver GT is very rare. A few should be uncertain malignant potential tumors, so the patient should receive long-term clinical follow-up.

Financial support and sponsorship

This study was supported by grants from the Project of Medical and Health Technology Program in Zhejiang Province, China (No. 2022KY1185).

Conflicts of interest

There are no Conflicts of interest.

   References Top

Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: Analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol 2001;25:1-12.  Back to cited text no. 1
Jaiswal VR, Champine JG, Sharma S, Molberg KH. Primary glomangioma of the liver: A case report and review of the literature. Arch Pathol Lab Med 2004;128:e46-9.  Back to cited text no. 2
Gassel HJ, Klein I, Timmermann W, Kenn W, Gassel AM, Thiede A. Presentation of an unusual benign liver tumor: Primary hepatic glomangioma. Scand J Gastroenterol 2002;37:1237-40.  Back to cited text no. 3
Geramizadeh B, Nikeghbalian S, Shamsaifar A, Kazemi K, Tavoosi H, Sefidbakht S, et al. Primary glomus tumor of the liver: A rare case report and review of the literature. Indian J Pathol Microbiol 2011;54:584-7.  Back to cited text no. 4
[PUBMED]  [Full text]  
Li L, Xu QX, Zhang XY, Han CH. Unusual location of the glomus tumour in the liver: A case report and literature review. Medicine (Baltimore) 2018;97:e11294.  Back to cited text no. 5
Hirose K, Matsui T, Nagano H, Eguchi H, Marubashi S, Wada H, et al. Atypical glomus tumor arising in the liver: A case report. Diagn Pathol 2015;10:112.  Back to cited text no. 6

Correspondence Address:
Rong Ge
Department of Diagnosis, Ningbo Diagnostic, Pathology Center, Ningbo - 315 021
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_415_21

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