Xanthogranulomatous inflammation is a chronic inflammation whose diagnosis remained a dilemma for gynecologists and pathologists. Its involvement of female genital tract is rare and involves ovaries, tubes, uterus and cervix. Etiopathogenesis is not clear and hypothesis like infective etiology, radiation, cancer, etc., have been proposed. Clinical symptoms and signs may vary depending upon location of this inflammation like abdominal pain, swelling, fever, purulent discharge, tub ovarian mass, etc., USG and CECT pelvis may show solid cystic tub ovarian mass, uterine collection and swelling, etc. Patients with pyometra or having infective etiology may respond to antibiotic, but who have large tub ovarian mass need surgery. Diagnosis is confirmed by histopathology.

Keywords: Xanthogranulomatous inflammation, Female genital tract

Abbreviations: XGI: Xanthogranulomatous Inflammation

Xanthogranulomatous inflammation (XGI) is a rare form of chronic inflammation that is characterized histopathologically by a marked proliferative fibrosis, parenchymal destruction, and infiltration of foamy histiocytes mixed with hemosiderin laden macrophages and foreign body giant cells. It has been reported in multiple organs, most commonly in the kidney, gall bladder, salivary glands and bones. It is less commonly seen in the female genital tract [1-3].

DISCUSSION

Various other terms have also been used like pseudoxanthoma and histolytic endometritis. Histolytic endometritis was coined by Buckley and Fox in 1980 [4]. Malakoplakia is another rare variant of histiocytic endometritis which shows Michaelis-Gutmann bodies, i.e., intra and extracellular calcified spherules. Immunohistochemistry for xanthogranulomatous inflammation is positive for Vimentin and CD 68.

LOCATION

1. Adnexa including ovaries and tubes as tub ovarian mass

2. Uterus including myometrium, perimetrium [5] and as xanthogranulomatous endometritis [6]

3. Cervix

Etiopathogenesis 

Although the exact etiology of the disease is not known, however, various theories have been proposed in literature. Suppurative infections, organ obstruction and hemorrhage trigger leads to tissue damage within the involved organs, usually eliciting a microscopic response. XGI causes destruction of the involved organ and could be misinterpreted as a locally invasive cancerous lesion. Biedermann et al. [7] supports the Infective etiology theory as long-standing infections with mycoplasma hominis in a patient  of  tubo-ovarian abscess leads to peritonitis. Various other organisms have been isolated from the bacterial culture such as E. coli, Proteus spp., Staphylococcus aureus, Bacteriodes fragilis and Salmonella typhi. Inoue et al. [5] reported the case of XGI of perimetrium, myometrium and endometrium because of abscess from the perimetrium and infiltrating deep into the posterior uterine myometrium which also supports the infective etiology. XGI may be influenced by various factors like tumor tissue or dead tumor cells due to necrosis, presence of abundant amount of intrauterine hemorrhage and cervical stenosis [8]. XGI associated with post-menopausal pyometra due to cervical stenosis or as a result of cervical carcinoma has also been reported. Theory of endometriosis, intrauterine contraceptive device, inborn errors of lipid metabolism and drug have also been proposed. Endometrial carcinoma that had been irradiated with external beam and/or intracavitary implants is also associated with xanthogranulomatous inflammation. Similarly, xanthogranulomatous cervicitis has been reported in a patient of cervical cancer in specimen of radical hysterectomy who was treated initially with external beam irradiation for the cervical cancer [9]. Uterine artery embolisation may also predispose this condition causing ischaemia or chronic obstructive process leading to congestion and infection [10]. XGI with ovarian hemangioma [11], diverticulitis [12], uterine leiomyoma [13] association with diabetes mellitus [14], talcum powder [15] and as an unusual complication of typhoid [16] has also been reported (Figure 1).

Age

It has been reported in every age group from prepubertal to postmenopausal. Tanwar et al. [17] reported XGI in a 2 year female child with right-sided xanthogranulomatous salpingooophoritis presented as mass in abdomen. Although the juvenile form is often considered to undergo regression; the adult xanthogranuloma is persistent in most of the cases [18]. The juvenile form has been described with hematologic malignancies such as B-cell acute lymphoblastic leukemia [19].

Presentation

1.      Guzman-Valdivia G (2004) Xanthogranulomatous cholecystitis: 15 years’ experience. World J Surg 28: 254-257.

2.      Ladefoged C, Lorentzen M (1988) Xanthogranulomatous inflammation of the female genital tract. Histopathology 13: 541-51.

3.      Singh A, Vats G, Radhika AG, Meena P, Radhakrisnan G (2016) Cervical xanthogranuloma in a case of postmenopausal pyometra. Obstet Gynecol Sci 59: 411-414.

4.      Buckley CH, Fox H (1980) Histiocytic endometritis. Histopathology 4: 105-110.

5.      Inoue T, Oda K, Arimoto T, Samejima T, Takazawa Y, et al. (2014) Xanthogranulomatous inflammation of the perimetrium with infiltration into the uterine myometrium in a postmenopausal woman: A case report. BMC Womens Health 15: 82.

6.      Makkar M, Gill M, Singh D (2013) Xanthogranulomatous endometritis: An unusual pathological entity mimicking endometrial carcinoma. Ann Med Health Sci Res 3: 48-49.

7.      Biedermann L, Schaer DJ, Montani M, Speich R, Mullhaupt B (2009) Extensive chronic xanthogranulomatous intra-abdominal inflammation due to Mycoplasma hominis mimicking a malignancy: A case report. J Med Case Rep 3: 9211.

8.      Pounder DJ, Iyer PV (1985) Xanthogranulomatous endometritis associated with endometrial carcinoma. Arch Pathol Lab Med 109: 73‑75.

9.      Govindaraman PK, Marimuthu S (2016) A rare case of xanthogranulomatous cervicitis in post radiation radical hysterectomy. Int J Curr Res Med Sci 4: 3094-3096.

10.   Singh N, Tripathi R, Mala YM, Arora S (2013) Xanthomatous oophoritis following uterine artery embolisation: Successful conservative surgical management with favourable outcome. BMJ Case Rep 4: 2013.

11.   Shashikala K, Sharmila PS, Sushma TA, Francis P (2013) Ovarian haemangioma with synchronous xanthogranulomatous inflammation - A rare pathological finding. Int J Health Sci Res 5: 116-119.

12.   Altanis S, Raweily E, Katesmark M (2007) Xanthogranulomatous endometritis and oophoritis secondary to diverticulitis. A rare cause of postmenopausal bleeding. J Obstet Gynaecol 27: 746-747.

13.   Abeysundara PK, Padumadasa GS, Tissera WG, Wijesinghe PS (2012) Xanthogranulomatous salpingitis and oophoritis associated with endometriosis and uterine leiomyoma presenting as intestinal obstruction. J Obstet Gynecol Res 38: 1115-1117.

14.   Chou SC, Wang JS, Tseng HH (2002) Malacoplakia of the ovary, fallopian tube and uterus: A case associated with diabetes mellitus. Pathol Int 52: 789-793.

15.   Chouairy CJ, Hajal EA, Nehme YA (2012) Xanthogranulomatous oophoritis secondary to talcum powder. Case report and review of the literature. J Med Liban 60: 169-172.

16.   Singh UR, Revathi G, Gita R (1995) Xanthogranulomatous oophoritis: An unusual complication of typhoid. J Obstet Gyaecol 21: 433-436.

17.   Tanwar H, Joshi A, Wagaskar V, Kini S, Bachhav M (2015) Xanthogranulomatous salpingooophoritis: The youngest documented case report. Case Rep Obstet Gynecol 2015: 237-250.

18.   Hwang SH, Son EJ, Oh KK, Kim EK, Jung JJ, et al. (2007) Bilateral xanthogranuloma of the breast radiologic findings and pathologic correlation. J Ultrasound Med 26: 535-537.

19.   Shoo BA, Shinkai K, McCalmont TH, Fox LP (2008) Xanthogranulomas associated with hematologic malignancy in adulthood. J Am Acad Dermatol 59: 488-493.

20.   Wader JV, Jain A, Kumbhar SS, Vhawal V (2013) Histiocytic endometritis. Am J Case Rep 14: 329-332. 

21.   Noack F, Briese J, Stellmacher F, Hornung D, Horny HP (2006) Lethal outcome in xanthogranulomatous endometritis. APMIS 114: 386-388.