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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:sadimani243@gmail.comMob 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)
Saranya.B, et.al : Xanthogranulomatoussalphingitis and pseudoxanthomatoussalphingitis in a patient
212National Journal of Basic Medical Sciences | Volume 8 | Issue 4 | 2018
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
Saranya.B, et.al : Xanthogranulomatoussalphingitis and pseudoxanthomatoussalphingitis in a patient
213National Journal of Basic Medical Sciences | Volume 8 | Issue 4 | 2018
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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Saranya.B, et.al : Xanthogranulomatoussalphingitis and pseudoxanthomatoussalphingitis in a patient
Received on : 18.04.2018 Revised on : 02.05.2018 Accepted on: 05.06.2018