Kidney Disease: Glomus Tumor Of The Kidney
Mar 17, 2022
Contact: Audrey Hu Whatsapp/hp: 0086 13880143964 Email: audrey.hu@wecistanche.com
Primary glomus tumor of the kidney
K.R. Aarthiprabha, Saloni Naresh Shah, Maya Menona, S. Annapurneswarib
Abstract: Primary glomus tumors of the kidney are rare with only 33 cases reported in the literature. We present a 59-year-old lady with abdominal pain and dyspepsia with a provisional diagnosis of renal cell carcinoma on imaging. Pathological examination revealed a mesenchymal pericytic tumor with morphology and immunohistochemistry being most consistent with malignant glomus tumor. We believe this is the first case of glomus tumor of the kidney in the Indian literature. We report this case given its rarity and to add to the existing published data.
cistanche treating kidney diseases
Introduction
Glomus tumors are mesenchymal neoplasms classified as pericytic (perivascular) tumors by the world health organization (WHO). They constitute less than 2% of all soft tissue tumors, of which a quarter arises from the visceral organs. (Lamba et al.,2011)They originate from neuro myoarterial glomus bodies situated in the cutis and superficial soft tissues and present as painful subungual nodules. (Li et al.,2017). Glomus tumors have been described in visceral organs such as lung, liver, gastrointestinal tract, bone, female genital tract, mediastinum, and genitourinary tract, despite their apparent absence at these sites. (Lamba et al,2011; Chen et al,2017;Al-Ahmadie et al,2007)Renal glomus tumors are rare with only 33 reported cases to date worldwide. (Dee et al.,2018)These patients may present with hypertension, flank pain, microscopic hematuria, as any other renal tumor and cannot be distinguished from renal cell carcinoma radiologically. Therefore, pathological evaluation is the sine qua non for a definite diagnosis. We present the first case of Glomus tumor of kidney from India with a review of the literature and a detailed discussion on the differential diagnoses.
cistanche treating kidney diseases
Case report
A 59-year-old lady presented with complaints of vague abdominal pain and dyspepsia to our hospital. She was a known hypertensive with a history of left radical nephrectomy, performed elsewhere 14 years ago. The patient had no records of her previous hospitalization. On abdominal examination, a non-tender mass measuring 6×6 cm was noted in the midclavicular line 6 cm below the right costal margin. Imaging revealed an exophytic mass at the lower pole of the right kidney. A partial nephrectomy was performed with a provisional diagnosis of renal cell carcinoma. On gross examination, pale white, lobulated, soft to firm, hemorrhagic mass with irregular borders and a thin capsule measuring 7.5×7.5×6 cm was noted. A sleeve of normal renal parenchyma was present at the surgical margin. Histopathological examination revealed a solid epithelioid neoplasm with cells arranged as diffuse sheaths and in perivascular pattern with numerous interspersed dilated blood vessels of varying sizes [Fig. 1A-C]. The cells had moderate eosinophilic cytoplasm, round to ovoid nucleus, and small nucleoli [Fig. 1D and E]. Scattered many highly pleomorphic cells with an enlarged nucleus and eosinophilic nucleoli [Fig. 1F and G] and patches of necrosis[Fig. 1H]were also present. Up to 5 mitoses were seen per 50 HPF [Fig. 1D-F]. Capsular infiltration was present with a few entrapped tubules at the periphery of the lesion [Fig. 1I]. Immunohistochemistry showed diffuse positivity for vimentin, smooth muscle actin (SMA), h-caldesmon, and CD34 in the epithelioid cells and was negative for pan-cytokeratin, desmin, HMB45, S-100 and CD117 [Fig. 2]. The morphology and immunohistochemistry were consistent with the malignant glomus tumor of the kidney. The patient was doing well two years post-surgery with no evidence of recurrence or metastasis.
cistanche can anti-tummor and cancer
Discussion
Glomus tumors of the kidney are extremely uncommon with 27 benign glomus tumors, 3 cases of glomus tumors of uncertain malignant potential (GT-UMP) and 3 malignant glomus tumors that have been reported to date.(Lamba et al.,2011;Li et al.,2017;Chen et al,2017;Al-Ahmadie et al.,2007; Dee et al.,2018; Sirohi et al.,2017;Folpe et al. 2001; Gill and Van Vliet,2010)Patients ranged from 17 to 81 years of age with slight male preponderance. Most of these tumors were exophytic and polar, with an occasional tumor arising from the ureteropelvic junction. These were well-circumscribed with areas of hemorrhage and necrosis and ranged from 1 to 16 cm in size. Based on the histological features the variants can be designated as glomus tumor, glomangiomas, or gloman giomyomas. In 2001, Folpe and colleagues proposed the histological criteria for malignant glomus tumors of the soft tissue: large size(>2cm) and deep location (or) atypical mitotic figures (or)marked atypia with 5 or more mitoses per high power field. (Folpe et al.,2001)In this regard, our case fulfilled the criteria for malignancy. However, in subsequent years some authors believed that these criteria may not apply to renal glomus tumors, as most of the cases in the literature were large-sized and in deeper locations but had a benign clinical course. (Li et al.,2017;Sirohi et al.,2017) Malignant transformation is seen in 5% of the cases with large size of the tumor, nuclear pleomorphism, presence of necrosis, increased/atypical mitoses, and lymphovascular invasion being the predictors of the metastatic potential. (Li et al., 2017)
Fig 1. Renal tumor arranged in a perivascular pattern with an adjacent normal renal parenchyma(A, B, C)composed of epithelioid cells with pleomorphism and scattered mitotic figures (D to G), foci of necrosis,and focal capsular infiltration(Haematoxylin&Eosin stain).
Fig 2. Immunohistochemistry stain showing positivity for Vimentin, SMA,h- Caldesmon, and CD34, and negativity for Pan CK, c-KIT, desmin, and S-100.
Immunohistochemically, glomus tumors are positive for vimentin, smooth muscle actin (SMA), and pericellular type IV collagen. (Li et al. 2017; Chen et al,2017)19% show CD34 positivity.(Folpe et al.2001)p53 is prominently expressed in malignant glomus tumors as compared to the benign counterpart.(Gill and Van Vliet,2010) Mesenchymal tumors such as epithelioid angiomyolipoma, epithelioid leiomyoma, hemangiopericytoma, juxtaglomerular cell tumor(JGCT), epithelioid solitary fibrous tumor (SFT), paraganglioma, carcinoid tumor, and renal cell carcinoma warrant exclusion before arriving at a final diagnosis. Epithelioid variant of angiomyolipoma belonging to the PEComa group is a benign neoplasm composed of a triad of thick-walled blood vessels, proliferating smooth muscle cells and adipocytic cells in varying pro-portions along with a predominant population of polygonal cells with dense eosinophilic cytoplasm, large vesicular nucleus, and prominent nucleoli which express HMB45 and Melan A. Leiomyomas are composed of smooth muscle bundles with desmin positivity, lacking the classic perivascular pattern of glomus tumor. (Dee et al.,2018)SFT is usually well circumscribed with the proliferation of bland spindle cells in patternless in a hyalinized stroma without mitotic figures, necrosis, or cystic change. The cells are strongly positive for STAT6 and Bcl2 and negative for smooth muscle markers as seen. (Al-Ahmadie et al,2007) Paraganglioma is composed of uni-form monomorphic cells arranged in a nested or zellballen pattern sur-rounded by spindled sustentacular cells and blood vessels, where the cells are positive for synaptophysin and chromogranin and the sustentacular cells express S100. Carcinoids show trabaculae of round and monomorphic cells with stippled nuclear chromatin and express neuroendocrine markers like synaptophysin and chromogranin. (Dee et al, 2018)Juxtaglomerular cell tumor (JGCT) is composed of monomorphic nested round cells with entrapped normal tubules and rhomboid-shaped crystal on electron microscopy. The neoplastic cells are positive for renin, vimentin, and CD34 and are negative for myoid markers. Both benign and malignant glomus tumors show MIR143-NOTCH gene fusion. Gene fusions involving MIR143 in band 5q32 and NOTCH2 in lp13(malignant glomus tumor) or NOTCH1 in 9q34 (benign glomus tumor) are seen in 50% of cases. (Sirohi et al.,2017)
cistanche supplement improve immunity
Conclusion
Primary glomus tumors of the kidney though rare, are mostly benign with only three malignant cases in the literature. The first case presented with multiple osseous metastases in addition to the malignant morphology of the renal tumor. Despite first-line chemotherapy and radiation, the patient died six months post-diagnosis. (Lamba et al.,2011)The second case had metastatic disease with multiorgan involvement 7 years after primary diagnosis of renal glomus tumor. The third case was an organ-confined glomus tumor of the kidney with features consistent with malignancy.(Chen et al.,2017)Our case was a localized primary kidney tumor with perinephric infiltration, large size (7.5cms)with cellular pleomorphism and increase mitoses, predicting its aggressive behavior thus favoring malignancy. Pathological examination is extremely crucial and remains the mainstay for arriving at a definitive diagnosis. Complete excision of the tumor by partial or radical nephrectomy is the preferred modality of treatment.(Chen et al.,2017) Given the history of left radical nephrectomy and the exophytic location of this right kidney tumor, a nephron-sparing surgery with adequate margin was performed in our patient. Prognosis, recurrence rate, and metastatic potential of glomus tumor of the kidney are yet to be determined due to the dearth of these neoplasms.
Patient consent statement
The patient's consent was obtained before the surgery.
Declaration of Competing Interest
On behalf of all authors, the corresponding author states that there is no conflict of interest.
Credit authorship contribution statement
K.R.Aarthiprabha: Conceptualization, Methodology, Data curation, Writing-original draft. Saloni Naresh Shah: Conceptualization, Methodology, Supervision, Data curation, Writing-original draft, Writ-ing-review & editing, Project administration.Maya Menon: Writing-review & editing. S. Annapurneswari: Writing-review & editing.
Acknowledgment
Nil
Supplementary materials
Supplementary material associated with this article can be found, in the online version, at doi:10.1016/j.cpccr.2021.100102.
cistanche improves immunity
References
Al-Ahmadie, H.A,Yilmaz,A.,Olgac,S.Reuter, V.E.2007. Glomus tumor of the kidney: a report of 3 cases involving renal parenchyma and review of the literature. Am. J Surg, Pathol 31.585-591.
Chen, Y.-A.,Li, H.-N., Wang, R.-C. Hung, S.-W., Chiu, K.-Y., 2017.Malignant glomus tumor of the kidney; a case report and review of the literature. Clin, Genitourin. Cancer 15,151-153.
Dee,E,Loghin.A. Toth, T,Naznean, A.,Borda,A.,2018. Glomus tumor of the kidney: case report. Acta Med. Marisiensis 64.126-129.
Folpe,A.L, Fanburg-Smith, J.C., Miettinen, M,Weiss,S.W.,2001.Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am. J.Surg.Pathol. 25, 1-12.
Gill, J., Van Vliet, C.,2010. Infiltrating glomus tumor of uncertain malignant potential arising in the kidney. Hum. Pathol. 41, 145-149.
Lamba, G., Rafivath, S.M., Kaur, H, Khan.S., Singh. P, Hamilton, AM, et al..2011. Malignant glomus tumor of the kidney: the first reported case and review of the literature. Hum. Pathol. 42,1200-1203.
Li, R.,Petros, F.G,Davis, CJ.,Ward, J.F.,2017.Characterization of glomus tumors of the kidney. Clin. Genitourin. Cancer 8 (16), 253-256.
Sirohi,D., Smith, S.C. Epstein, J.I, Balzer, B.L.,Simko, J.P., Balitzer, D.,et al,2017.Pericytic tumors of the kidney-a clinicopathologic analysis of 17 cases. Hum. Pathol. 64,106-117.
