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  Table of Contents    
CASE REPORT  
Year : 2011  |  Volume : 54  |  Issue : 3  |  Page : 584-587
Primary glomus tumor of the liver: A rare case report and review of the literature


1 Department of Pathology, Transplant Research Center, Shiraz University of Medical Sciences, Iran
2 Department of Surgery and Transplantion, Shiraz University of Medical Sciences, Iran
3 Department of Surgery, Mashhad University of Medical Sciences, Iran
4 Department of Radiology, Shiraz University of Medical Sciences, Iran

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Date of Web Publication20-Sep-2011
 

   Abstract 

Glomus tumor is a rare neoplasm of the distal extremities. It occurs very rarely in the deep visceral organs such as stomach, lung, pancreas, ovary, and liver. Herein, we report a very rare case of glomus tumor of the liver in a 50-year-old woman presenting with abdominal mass which was diagnosed after surgery. The tumor was large and cystic; however, the morphology was similar to the ordinary soft-tissue glomus tumor. Tumor cells were reactive with CD34 and SMA. The patient's follow up failed to show any evidence of malignant behavior or tumor recurrence. This report is the third primary glomus tumor of the liver in the English literature.

Keywords: Glomus tumor, liver, primary

How to cite this article:
Geramizadeh B, Nikeghbalian S, Shamsaifar A, Kazemi K, Tavoosi H, Sefidbakht S, Malek-Hosseini SA. Primary glomus tumor of the liver: A rare case report and review of the literature. Indian J Pathol Microbiol 2011;54:584-7

How to cite this URL:
Geramizadeh B, Nikeghbalian S, Shamsaifar A, Kazemi K, Tavoosi H, Sefidbakht S, Malek-Hosseini SA. Primary glomus tumor of the liver: A rare case report and review of the literature. Indian J Pathol Microbiol [serial online] 2011 [cited 2014 Aug 28];54:584-7. Available from: http://www.ijpmonline.org/text.asp?2011/54/3/584/85101



   Introduction Top


Glomus organs are arteriovenous anastomotic structures which control thermoregulation of the extremities. [1] The individual glomus cells have ultrastructural features of modified smooth muscle cells. [2] Glomus tumors are rare mesenchymal neoplasms, composed of cells with close resemblance to glomus cells. [3]

Glomus tumors usually present as subcutaneous masses of the distal extremities, especially in the subungual area. Rare cases have been reported in the deep soft tissue and viscera such as lung, [4] kidney, [3] ovary, [5] and stomach. [6] Glomus tumor of the liver is extremely rare and to the best of our knowledge, less than five cases have been reported in the English literature. [1],[2],[7],[8]

Herein, we report glomus tumor of the liver in a 50-year-old woman presenting with abdominal pain.


   Case Report Top


A 50-year-old woman presented with chief complaint of diffuse abdominal pain of 6 months' duration. The patient was well with no significantly abnormal physical findings, except for right upper quadrant bulging. Heart and lung examination were normal. Complete blood count was normal (WBC = 5 600/ml with normal differential, RBC = 5 Χ 10 6 /ml, Hb = 13 gr/dl). Biochemical tests were also normal (BUN = 20 mg/dl, Cr = 1 mg/dl, AST = 23 IU/l, ALT = 28 IU/l, and Alk-P = 86 IU/l). Blood sugar, cholesterol, and triglycerides were in normal range. All the tumor markers including CEA, AFP, CA19-9, and CA-125 were within normal limit.

Abdominal sonography revealed a large cystic mass in the liver. The patient underwent a dual-phase helical computed tomographic examination of the abdomen and pelvis which revealed a 15-cm hypervascular mass in the left lobe of liver [Figure 1], with cystic components and fluid/fluid levels indicating intralesional bleeding and hematocrit effect. It also showed multiple enhancing septa. The hepatic vein was displaced and the hepatic artery left lobar branch was enlarged. The IVC was compressed but not invaded.
Figure 1: CT scan shows a huge hypervascular hepatic tumor

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With the impression of vascular tumor, the patient underwent an exploratory laparotomy in the local hospital, but the mass was so large that the general surgeon decided not to resect it; therefore, a tissue biopsy was taken and the patient was referred to a hepatobiliary surgeon for evaluation of the possibility of tumor excision. The biopsy showed hypervascular tumor and the differential diagnoses of hemangioma or hemangioendothelioma, to be definitely diagnosed after complete excision of the mass.

An exploratory laparotomy was performed in our center to remove the hepatic mass. The peritoneal cavity was entered through Mercedes incision. After mobilization of the liver and ligation of vessels and cholecystectomy, left hepatic lobectomy was performed. The postoperative course was uneventful and now after a year, she is doing well and symptom-free.

Pathologic Findings

The specimen consisted of a 15 x 9 x 3-cm mass with thin rim of normal liver parenchyma around it. The mass was mostly cystic and filled with fresh blood [Figure 2]. Light microscopic examination of the mass showed sheets of small uniform round to oval cells, separated by thin fibrovascular stroma [Figure 3]a. Individual tumor cells showed moderate eosinophilic cytoplasm with distinct cytoplasmic borders [Figure 3]b. The nuclear borders were smooth and regular with open chromatin. No atypia and no mitosis were identified. Immunohistochemistry (IHC) of the tumor cells showed reactive smooth muscle actin (SMA) and CD34 [Figure 3]c and d. CD34 was positive in both endothelial and myoid cells. CD31 reactivity was limited to the endothelial cells. All the other markers, including S100, HMB45, AE1-AE3, CD10, Bcl2, EMA, CEA, chromogranin, NSE, desmin, CD99, and c-kit, were negative. The proliferation index was <1% by IHC staining for Ki-67.
Figure 2: The cut surface of the liver glomus tumor

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Figure 3: (a) Low power microscopic view of the glomus tumor (H and E x250), (b) High-power view shows uniform cells with distinct cytoplasmic border, without mitosis or atypia (H and E x400), (c) SMA reactivity in the myoid cells (IHC x400), (d) CD34 reactivity in both the endothelial and myoid cells (IHC x250)

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The patient was diagnosed as having glomus tumor (glomangiomyoma).

The patient was discharged in good health condition and now after about 12 months, she is completely symptom-free, without any tumor recurrence in imaging studies.


   Discussion Top


The families of glomus tumors are composed of a group of tumors with a mixture of glomus cells, blood vessels, and smooth muscle cells in different proportions. [3]

These tumors represent 0.012% of all tumors diagnosed in human being. [4] Most of these cases present as subcutaneous masses of distal extremities, but have also been reported in other sites such as gastrointestinal tract, [9] bone, [10] ovary, [5] lung, [4] stomach, [6] and pancreas. [11] Glomus tumor of the liver is an extremely rare tumor. To the best of our knowledge, only two other cases have been reported in the English literature. [1],[2],[7],[8] Both of the previous cases were in the patients above 50 years of age, similar to our case (50-year-old). The clinical and pathologic findings of the two previously reported cases and our patient are shown in [Table 1]. The most unique and significant difference between our tumor and previous ones is the large size of our tumor (15 Χ 9 Χ 3 cm).
Table 1: Main characteristics of the 3 reported cases of liver glomus tumor

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In all the three cases, histopathology failed to show any evidence of anaplasia. The main differential diagnoses in this hepatic mass were hemangioendothelioma, metastatic gastrointestinal stromal tumor, PECOMA (pericytoma), and endocrine tumors. Presence of uniformly small, round to polygonal cells without mitosis or atypia exclude malignant vascular tumors and also absence of intracytoplasmic lumina was against the diagnosis of hemangioendothelioma. [2] Concomitant reactivity of tumor cells for SMA, CD 34, and lack of reactivity for c-kit help to distinguish this tumor from metastatic GIST(gastrointestinal stromal tumor). Meanwhile, upper and lower endoscopy as well as endosonography failed to show any evidence of GI submucosal tumor. Negative HMB 45 and desmin were against the diagnosis of PECOMA. [12]

At last, negative endocrine markers (Chromogranin, synaptophysin, and NSE) exclude carcinoid and other endocrine tumors. [2]

Another important consideration in these tumors is their prognosis. Glomus tumors are generally considered benign, and malignant cases are exceedingly rare. [3]

Recently, criteria of malignancy were proposed as the following:

1, 2, 3 - moderate to marked nuclear atypia, 4 - ≥5 mitotic figures/50 HPF. [13] The present case is thought to be an exception, although there was no atypia or mitosis, despite of the absence of nuclear labeling by Ki-67, but its large size (15 cm) was indicative of malignant behavior. However, until now (after 12 months of follow-up), the patient is well and symptom-free with no evidence of tumor recurrence in the imaging studies.

 
   References Top

1.Gassel HJ, Gassel AM, Marx A, Kenn W, Scheurlen M, Timmermann W, et al. Primares glomangiom der leber. Chirurg 2002;73:279-82.  Back to cited text no. 1
[PUBMED]  [FULLTEXT]  
2.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
[PUBMED]  [FULLTEXT]  
3.Al-Ahmadie HA, Yilmaz A, Olgac S, Reuter VE. Glomus tumor of the kidney: A report of 3 cases involving renal parenchyma and review of the literature. Am J Surg PAthol 2007;31:585-91.  Back to cited text no. 3
[PUBMED]    
4.Kapur U, Helenowski M, Zayaad A, Ghai R, Vigeneswaran W, Rajan P. Pulmonary glomus tumor. Ann of Diagn Pathol 2007;11:457-9.  Back to cited text no. 4
    
5.Slone SP, Moore GD, Parker LP, Rickard KA, Nixdorf-Miller AS. Glomus tumor of the ovary masquerading as granulosa cell tumor: Case Report. Int J Gynecol Pathol 2010;29:24-6.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Miettinen M, Paal E, Lasota J, Sobin LH. Gastrointestinal glomus tumor, a clinicopathologic, immunohistochemical, and molecular genetic study of 32 cases. Am J Surg Pathol 2002;26:301-3.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  
7.Kenn W, Klein I, Gassel HJ, Gattenloehner S, Gassel AM, Hahn D. Primary glomangioma of the liver: Imaging findings. Abdom Imaging 2002;27:716-9.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.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. 8
[PUBMED]    
9.Haque S, Modlin IM, West AB. Multiple glomus tumors of the stomach with intravascular spread. Am J Surg Pathol 1992;16:291-9.  Back to cited text no. 9
[PUBMED]    
10.Rozmaryn LM, Sadler AH, Dorfman HD. Intraosseous glomus tumor in the ulna. A case report. Clin Orthop Relat Res 1987;220:126-9.  Back to cited text no. 10
[PUBMED]    
11.Miliauskas JR, Worthley C, Allen PW. Glomangiomyoma (glomus tumour) of the pancreas: A case report. Pathology 2002;34:193-5.  Back to cited text no. 11
    
12.Mitteldorf CA, Birolini D, da Camara-Lopes LH. A perivascular epithelioid cell tumor of the stomach: An unsuspected diagnosis. World J Gastroenterol 2010;16:522-5.   Back to cited text no. 12
    
13. Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. A typical 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. 13
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Correspondence Address:
Bita Geramizadeh
Department of Pathology, Transplant Research Center, Shiraz University of Medical Sciences, Shiraz, PO Box: 71345-1864
Iran
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DOI: 10.4103/0377-4929.85101

PMID: 21934228

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    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
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