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CASE REPORT  
Year : 2020  |  Volume : 63  |  Issue : 3  |  Page : 467-469
Cervical rhabdomyoma a rare entity: Case report with review of literature


1 Department of Pathology, Maulana Azad Medical College, New Delhi, India
2 Department of Gynaecology, Maulana Azad Medical College, New Delhi, India

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Date of Submission28-Jan-2019
Date of Decision23-Jul-2019
Date of Acceptance26-Jul-2019
Date of Web Publication7-Aug-2020
 

   Abstract 


Genital rhabdomyomas are extremely rare benign tumors of skeletal muscle origin, majority of them being reported in vaginal location. Extensive literature search revealed only three such cases reported in cervix. We hereby report fourth such case of cervical rhabdomyoma in a 35-years-old female patient. The diagnosis was confirmed by histomorphology with desmin and myoD1 positivity on immunohistochemistry. Due to paucity of cases no definite treatment guidelines are available. Differentiation from identical and more common malignant counterpart which is rhabdomyosarcoma is essential to avoid unnecessary aggressive therapy.

Keywords: Cervical polyp, cervical rhabdomyoma, genital rhabdomyoma, myo D1, rhabdomyoblast

How to cite this article:
Sagar N, Mandal S, Khurana N, Kumar A. Cervical rhabdomyoma a rare entity: Case report with review of literature. Indian J Pathol Microbiol 2020;63:467-9

How to cite this URL:
Sagar N, Mandal S, Khurana N, Kumar A. Cervical rhabdomyoma a rare entity: Case report with review of literature. Indian J Pathol Microbiol [serial online] 2020 [cited 2020 Oct 31];63:467-9. Available from: https://www.ijpmonline.org/text.asp?2020/63/3/467/291686





   Introduction Top


Extracardiac rhabdomyomas are rare tumors, seen in head and neck region. Their occurrence in female genital region is rare with most cases being reported in the vagina. Cervical rhabdomyoma is an extremely rare tumor.[1] Extensive literature search has revealed only three reported cases in the literature.[2],[3],[4],[5] We hereby report the fourth such case of cervical rhabdomyoma in a 35-year-old female.


   Case History Top


A 35-year-old female presented with complaints of bleeding per vaginum associated with a protrusion of mass from the vaginal introitus since one year. On examination a polypoidal mass arising from ectocervix was noted. The mass was locally excised and sent to pathology department of our hospital. Grossly a poypoidal bit was submitted measuring 5 × 3 × 1 cm. Microscopic examination on H/E stained slides revealed tissue lined by stratified squamous epithelium with stroma composed of fibrocollagenous tissue along with many rhabdoid cells showing cross striation [Figure 1]a. These cells were of variable size with abundant cytoplasm, large vesicular nucleus with central to peripheral arrangement [Figure 1]b and [Figure 1]c. No mitosis, necrosis or atypia was noted. On immunohistochemistry these cells were positive for Desmin [Figure 1]d and MyoD, while negative for S100 CK and EMA. A confirmatory diagnosis of cervical rhabdomyoma was rendered. The patient is on follow up and following one-year post surgery has shown no signs of recurrence.
Figure 1: (a) H/E-stained slides revealed tissue lined by stratified squamous epithelium with stroma composed of fibrocollagenous tissue and prominent blood vessels (H and E, 200×). (b) variable size cells with abundant cytoplasm, large vesicular nucleus with central to peripheral arrangement (H and E, 600×). (c) Rhabdoid cells with peripheral nuclei scattered in fibrous stroma (H and E, 600×). (d) MyoD1 positivity in rhabdoblasts (Immunohistochemistry, DAB chromogen 600×)

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   Discussion Top


Rhabdomyomas are rare tumors and accounts for less than 2% cases showing skeletal muscle differentiation.[6] They can be associated with syndromes like tuberous sclerosis, down syndrome, etc. Depending upon their site of occurrence they are divided as cardiac and extra cardiac. Based on morphology extra cardiac rhabdomyomas are divided as adult, fetal, and genital type.[1],[6] Adult rhabdomyoma shows round to polygonal cells with abundant eosinophillic and vacuolated cytoplasm. Fetal type rhabdomyoma show oval to spindle cells present in stroma comprised of more mature skeletal muscle differentiation with less myxoid stroma or less skeletal muscle differentiation with abundant myxoid stroma. Genital rhabdomyoma are uncommon tumors seen in young to middle aged females (25–55 years). With less than 30 cases have been reported in vagina, mere three cases have been reported in the cervix so far vaguely in literature [Table 1].[2],[3],[4],[5] Out of three cases reported one case was published in non-English literature and others were part of various case series.
Table 1: Reported cases of cervical rhabdomyoma[2],[3],[4]

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Clinically majority of the cases of genital rhabdomyomas are assymptomatic and found incidentally with occasional cases presenting with symptoms like vaginal bleeding and dyspareunia. The present case had complaints of bleeding and protusion of a mass from the vaginal introitus. Grossly these tumors are generally small ranging in size from 2 to 3 cm however cases as large as 11 cm have been reported in literature.[1],[6] On external exaination areas of ulceration and hemorrhage are atypical findings in these cases. Cut section of the tumor is usually firm, rubbery with glassy areas. Microscopically these tumors are lined by epithelium of site of occurrence with subepithelium showing haphazardly arranged rhabdomyoblastic cells with fascicles, present in variable amount of loose fibrocollagenous stroma. Myxoid areas may be seen at places. Rhabdomyoblasts are usually bland spindle to strap cells with abundant eosinophilic cytoplasm showing cross strations, large oval nucleus, and prominent eosinophilic nucleoli. Mitosis and nuclear atypia are unusual. Cambium layer is typically absent; a feature helps in differentiation from rhabdomyosarcomas. On immunohistochemistry these cells show positivity for desmin, muscle specific antigen, myogenin, myoD and negative for smooth muscle antigen, S100, cytokeratin and epithelial membrane antigen.[1],[6]

The differential diagnosis of cervical mass includes embryonal rhabdomyosarcoma (RMS), and leiomyoma. In contrast to RMS, rhabdomyomas show less mitotic activity and minimal nuclear pleomorphism. Cambium layer formation which is characteristic of embryonal rhabdomyosarcomas is absent in genital rhabdomyoma. Lack of cytoplasmic vacoulation and less variable stroma rules out adult and fetal rhabdomyoma, respectively.[1]

The pathogenesis of genital rhabdomyomas remains controversial. Some theories suggest it to be hamartomatous rather than true neoplasm.[7] However, if this was true, most cases would occur at a young age. On the contrary only 4 cases of genital rhabdomyomas have been reported in young children. The current acceptable hypothesis is that they originate from myogenic stem cells present in the stroma.[8]

Genital rhabdomyomas are treated with local excision with fertility preservation. Although local recurrence can be seen in rhabdomyomas especially the adult type, local recurrence, and metastasis has never been reported in genital rhabdomyomas.[4]

Genital rhabdomyoma of cervix is extremely rare entity which carries excellent prognosis with almost nil recurrence. Current treatment approach is fertility salvage local excision. Knowledge about histological and immunohistochemical feature is essential for correct diagnosis and differentiation from more common malignant counterpart in cervix.

Declaration of patient consent

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Lu DY, Chang S, Cook H, Alizadeh Y, Karam AK, Moatamed NA, et al. Genital rhabdomyoma of the urethera in an infant girl. Hum Pathol 2012;43:597-600.  Back to cited text no. 1
    
2.
Urbunke A. Reines Rhabdomyom der Gebarmutter. Zbl Allg Path 1962;103:241-3.  Back to cited text no. 2
    
3.
Iversen UM. Two cases of benign vaginal rhabdomyoma: Case reports. APMIS 1996;104:575-8.  Back to cited text no. 3
    
4.
Di Sant'Agnese PA, Knowles DM. Extracardiac rhabdomyoma: A clinicopathologic study and review of the literature. Cancer 1980;46:780-9.  Back to cited text no. 4
    
5.
Hunski W, Hagel-Lewicka E, Daniszewski K. Rhabdomyoma of female genital tract. Report on two cases. Zentralbl Pathol 1991;137:439-42.  Back to cited text no. 5
    
6.
Vasahar A. Genital Rhabdomyoma- A rare case report. Int Clin Pathol J 2017;4:90.  Back to cited text no. 6
    
7.
Lin GY, Sun X, Badve S. Pathologic quiz case: Vaginal wall mass in a 47-year-old woman. Arch Pathol Lab Med 2002;126:1241-2.  Back to cited text no. 7
    
8.
Hansen T, Katenkamp D. Rhabdomyoma of the head and neck: Morphology and differential diagnosis. Virchows Archiv 2005;447:849-54.  Back to cited text no. 8
    

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Correspondence Address:
Nishant Sagar
Department of Pathology, Maulana Azad Medical College, New Delhi - 110 002
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_73_19

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