xrare form of chronic inflammation which · ovarian cyst showed cyst wall lined by luteinised ......

A Very Rare Case of XanthogranulomatousSalphingitis in One Tube and

Pseudoxanthomatous Salphingitis in Contralateral Tube in a Patient

209

Case Report

1 2 3Saranya.B , Jayanthi Kamakshi.N , Thamilselvi.R

ABSTRACT

Xanthogranulomatous salphingitis and pseudoxanthomatous salphingitis are rare forms of chronic

inflammation of the fallopian tube with many etiologic factors implicated in its pathogenesis. A 26

year old female was operated for a right ovarian cyst. Ovarian cyst with bilateral fallopian tubes were

removed since both the fallopian tubes were dilated and adherent to the adjacent tissues. One tube

showed features of Xanthogranulomatous salphingitis and other pseudoxanthomatous salphingitis.

Ovarian cyst was a corpus luteal cyst. Although the exact etiology for this case was not clear,

endometriosis can be thought to have a contributory role in the pathogenesis as the patient had severe

dysmenorrhoea and severe adhesions were noted intraoperatively. Both Xanthogranulomatous

salphingitis and pseudoxanthomatous salphingitis were reported separately by many authors. But,

both these conditions in one patient to my knowledge is not yet reported in literature. Hence, this

case is reported for its rarity.

INTRODUCTION:

anthogranulomatous salphingitis is a

rare form of chronic inflammation which xcauses destruction to the normal tissue

of many organs. On histology, it is characterised by

the presence of numerous lipid containing

macrophages with an admixture of lymphocytes,

plasma cells and neutrophils. Multinucleated giant (1-4)

cells may be present . Kidney is the most

commonly affected organ followed by gall bladder.

Other organs affected are stomach, anorectal

region, bone, urinary bladder, testis and

epididymis. Female genital tract involvement is

relatively uncommon and it is mostly confined to

the endometrium. However, rare involvement of

vagina, cervix, fallopian tube and ovary have been (5-7) documented. As elsewhere in the body, the

mechanism of this form of inflammatory response

is not clear. Predisposing conditions for the

development of XGS include Pelvic Inflammatory

Disorder, the presence of an IUD, endometriosis,

and inborn error in lipid metabolism by

macrophages, unsuccessful antibiotic therapy, and

radiotherapy. Pseudoxanthomatous salphingitis is

another rare form of chronic inflammation

characterised by the presence of pigmented

histiocytes within the lamina propria. It is

associated with long standing endometriosis. Only

a few cases of Xanthogranulomatous salphingitis

and pseudoxanthomatous salphingitis have been

reported in literature separately by authors. But

Xanthogranulomatous sa lphingi t i s and

pseudoxanthomatous salphingitis in one patient is

not yet reported to the best of my knowledge.

Address for correspondence:

Dr.Saranya.B ,MayilIllam, Puduchatram , Namakkal , Tamilnadu - 637018 , India.

E- mail:[email protected] No. 9965655499

1 2 3Assistant Professor, Assistant Professor, Professor and HOD,Department of pathology, Vinayaka Missions Kirupanandavariyar Medical College and Hospitals,Vinayaka Missions Research Foundation - Deemed to be University, Salem , Tamilnadu – 636308.

National Journal of Basic Medical Sciences | Volume 8 | Issue 4 | 2018

http://dx.doi.org/10.31975/NJBMS.2018.8406

210

CASE REPORT :

A 26 year old female patient P L , both LSCS 2

presented to the Gynaecology department of

Vinayaka missions Kirupananda Variyar Medical

College and Hospitals with complaints of

abdominal pain on and off and white discharge per

vaginum. She had regular menstrual cycles with

severe dysmenorrhoea during cycles. There were

no history tuberculosis & contraceptive usage. On

examination, she had pallor. Per abdominal

examination was normal. Per speculum and per

vaginal examination revealed healthy cervix and

vagina except for white discharge. Ultra Sonogram

abdomen showed normal uterus and a cyst was

observed in the right ovary measuring 4 X 2 cms

with normal left ovary. Per operatively, extensive

adhesions were noted and hence adhesiolysis was

done. Since, both fallopian tubes were dilated and

adhered to the adjacent tissue, they were removed

along with the ovarian cyst. Ovarian cyst and both

fallopian tubes were sent for histopathological

examination.

PATHOLOGY:

Received both fallopian tubes in the same container

larger tube measuring 3 X 1.5 X 0.5 cms and

smaller tube measuring 2 X 2 X 0.5cms. Both tubes

were congested. C/S of larger tube showed dilated

and thinned out wall. C/S of the smaller tube

showed thickened wall filled with brownish

haemorrhagic material. Right ovarian cyst

measured 4.5 X 2.5 X 0.5 cms with irregular

congested external surface. C/S of the cyst was

irregular with trabeculations. Cyst wall showed no

solid areas/papillary excrescences. Section from

the larger tube showed mucosa thrown into folds

and lined by ciliated pseudostratified columnar

epithelium. Lamina propria showed sheets of

2

foamy macrophages and mild lymphocytic

infiltration. Section from the thickened tube

showed multiple fused plical folds forming follicle

like structures with lamina propria showing

aggregates of pigmented histiocytes, lymphocytes

and plasma cells. Few multinucleated giant cells

were also noted. Lumen of the smaller tube also

showed pigmented histiocytes. Sections from the

Ovarian cyst showed cyst wall lined by luteinised

granulosa layer and theca cell layer with

haemorrhage in the cyst.(Fig 1)

Hence, the diagnosis of xanthogranulomatous

salphingitis and pseudoxanthomatous salphingitis

with corpus luteal cyst in ovary was made.

DISCUSSION:

Xanthogranulomatous in f lammat ion i s

characterized by the presence of numerous, foamy,

lipid-rich histiocytes and other inflammatory (12)cells . This uncommon process is best known to

occur in the kidney. Xanthogranulomatous

inflammation has also been reported in the

gallbladder, stomach, anorectal area, bone, urinary

bladder, testis, epididymis, vagina, endometrium, (10)ovary, and fallopian tube .

A review of the literature revealed fourteen cases of (2, 10 –15)

fallopian tube involvement reported to date .

Patients with XGS ranged from 23 to 72 years old.

Initial presentation most often included lower

abdominal or suprapubic pain, fever, menorrhagia

and vaginal bleeding. Physical examination often (2,

showed a pelvic mass with adnexal tenderness14–18).

The exact pathogenesis of xanthogranulomatous

inflammation remains debated. Many unrelated

disorders may have the same mechanism of foam

cell production. Proposed causes are infection,

National Journal of Basic Medical Sciences | Volume 8 | Issue 4 | 2018

Saranya.B, et.al : Xanthogranulomatoussalphingitis and pseudoxanthomatoussalphingitis in a patient

211National Journal of Basic Medical Sciences | Volume 8 | Issue 4 | 2018

A. Gross picture of fallopain tubes. Pointed area shows thickened wall B. Gross picture of the ovarian cyst. Cyst wall shows trabeculations with no solid areas C. Corpus luteal cyst. Right lower area shows haemorrhage and left upper area shows luteinised Granulosa and theca cells(40 X) D. Thinned fallopian tube shows tubal plicae distended by xanthomatous cells (10 X) E. High power view of the xanthomatous cells (40 X) F. Thickened fallopian tube shows fused tubal plicae forming follicle like structures (Salphingtis follicularis) with inflammatory cells and pigmented histiocytes in the lamina propria (10 X) G.High power view of pigmented histiocytes (40 X)



ineffective antibiotic therapy, abnormality in lipid

metabolism, endometriosis, and ineffective

clearance of bacteria by phagocytes.

Predisposing conditions for the development of

XGS include PID, the presence of an Intrauterine

Device, endometriosis, and inborn error in lipid

metabolism by macrophages, unsuccessful (8–11)

antibiotic therapy, and radiotherapy. A whole

spectrum of changes may exist in this lesion and

probably represent a specialised form of tissue

reaction to multiple etiologies. For example,

bleeding and obstruction may predispose to

infection, and tissue necrosis may occur, followed

by the release of cholesterol and other lipids and

phagocytosis by macrophages.

Xanthogranulomatous salphingitis is often

misdiagnosed by pathologists if they don't keep this

entity in mind. This may be due to the rarity of the

c o n d i t i o n . D i f f e r e n t i a l d i a g n o s i s f o r

xanthogranulomatous salphingitis includenon

neoplastic and neoplastic conditions. Non

neoplastic conditions include tuberculous and

fungal infections which can be ruled out by doing

special stains and culture. For this case,

Ziehlneelsen stain and Periodic Acid Schiff stain

were negative. If the lesion contains mainly focal

scattered lymphocytes, it may be misdiagnosed as

secondary lymphoma or leukemia. If the

lymphocytes are scattered diffusely and foam cells

are seldom, adiagnosis of malignant small cell

tumor with stromal luteinization may be rendered.

If there are small amount of obvious fibrosis and

foam cells, a diagnosis of sclerosing stromal tumor (6,20)may be made . However, the right diagnosis is

possible to be made as long as pathologists elevate

vigilance and master the pathological features.

Immunohistiochemical stains are helpful in

establishing the diagnosis, including CD68 (foam

cells positive), CD3 (T lymphocyte marker), CD20

(B lymphocytes marker), κ and λ (both positive in (19)polyclonal B lymphocytes) .

( 2 0 )Walther presumed malakoplakia and

xanthogranulomatous inflammation were identical

chronic inflammatory disease. In malakoplakia, the

cytoplasmic concentric calcific bodies (Michaelis-

Gutmann bodies) are phagolysosome of

phagocytes which calcify after phagocytes

phagocytose E. coli but fail to digest it.

Malakoplakia occurs mainly in the urinary system,

but xanthogranulomatous inflammation occurs

mainly in the genital system.

Pseudoxanthomatous salpingiosis, which is an

equally rare phenomenon, occurs mainly in women

with a long history of endometriosis. PXS is

characterized by numerous pigmented histiocytes

containing brown lipofuscin (ceroid) pigment

within the lamina propria.

Interest ingly, we observed a different

histopathological finding in this case. These

included blunted and fused mucosal plicae forming

follicle like structures in tubes showing

pseudoxanthomatous salphingitis. This is a well

known entity called " Salphingitis follicularis".

This may be due to continuous surface fibrin

deposition that leads to permanent bridging (22)between mucosal folds .

Radiologically, Salphingitis follicularis may mimic

an adnexal neoplasm with cystic component.

Histopathologic examination of the material should

be done to make a final diagnosis. Another

differential diagnosis of these follicle like

structures is Salphingitis isthmica nodosa , which is

a diverticular disease of the fallopian tubes that



causes infertility. The etiology of this condition is

unknown. The possible explanations are post

inflammatory distortion and adenomyosis like

process. However in contrast to salphingitis

follicularis, this typically appears as yellow white

nodular swellings located at the isthmic portion of

the tube. Microscopically, they represent the

diverticula like structures that lie within the

hyperplastic smooth muscle with scant (23,24)surrounding inflammation .

Our patient presented with symptoms of severe

dysmenorrhoea and per operatively adhesions were

seen. The patient did not have any history of a lipid

metabolic disease nor her history was suggestive of

immunodefeciency. There was no evidence of

endometriosis histologically, however with the

history of dysmenorrhoea along with adhesions

seen peroperatively between fallopian tube and

adjacent structures, endometriosis can be

considered of as a contributory factor in the

pathogenesis of these conditions presenting as

xanthogranulomatous salphingitis in one tube and

pseudoxanthomatous salphingitis in the other tube.

CONCLUSION:

Xanthogranulomatous sa lphingi t i s and

pseudoxanthomatous salphingitis in one patient is a

very rare entity and we have described the possible

etiologic relationship of these conditions with

endometriosis. It should be borne in mind that both

xan thogranu lomatous sa lph ing i t i s and

pseudoxanthomatous salphingitis may cause

follicle like structures and may be pre operatively

diagnosed as adnexal neoplasm. Proper history,

r ad io log ica l examina t ion and p rec i se

histopathological examination may enlighten the

inflammatory pathology of the lesion and helps in

preventing misdiagnosis because of the rarity of

these conditions.

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Received on : 18.04.2018 Revised on : 02.05.2018 Accepted on: 05.06.2018

xrare form of chronic inflammation which · ovarian cyst showed cyst wall lined by luteinised ......

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