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   Table of Contents      
Year : 2005  |  Volume : 53  |  Issue : 1  |  Page : 57-59

Disseminated Metastasis following Periampullary Cancer Resection Masquerading as Uveitis

1 Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Cytopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Submission30-Apr-2003
Date of Acceptance17-Oct-2003

Correspondence Address:
Amod Gupta
Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0301-4738.15287

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The Uveitis Masquerade Syndromes (UMS) are a group of ocular diseases that mimic intraocular inflammation, but are in fact neoplastic in nature. We report a patient with disseminated malignancy who presented with uveitis 5 years after an apparently successful resection of periampullary adenocarcinoma. The Masquerade Syndrome was detected by cytological examination of the vitreous.

Keywords: Uveitis, Masquerade Syndrome, diagnostic vitrectomy, ocular metastasis

How to cite this article:
Kaushik S, Gupta V, Singh R, Vasishta RK, Rajwanshi A, Gupta A. Disseminated Metastasis following Periampullary Cancer Resection Masquerading as Uveitis. Indian J Ophthalmol 2005;53:57-9

How to cite this URL:
Kaushik S, Gupta V, Singh R, Vasishta RK, Rajwanshi A, Gupta A. Disseminated Metastasis following Periampullary Cancer Resection Masquerading as Uveitis. Indian J Ophthalmol [serial online] 2005 [cited 2020 Nov 30];53:57-9. Available from: https://www.ijo.in/text.asp?2005/53/1/57/15287

The Uveitis Masquerade Syndromes (UMS) are a group of ocular disorders that present as intraocular inflammatory processes, but are actually non-inflammatory diseases. Although some benign conditions can be considered masquerade syndromes, the term has conventionally been used for malignant entities.[1] The condition warrants urgent diagnosis, since the diseases that masquerade as uveitis are not only vision threatening, but may be potentially fatal. We report a patient who presented with UMS detected by a diagnostic vitrectomy 5 years after an apparently successful Whipple′s procedure for periampullary adenocarcinoma.

  Case report Top

A 60-year-old woman presented one-and-half years prior to writing, with a 6-month history of diminished vision in both eyes. The best corrected vision was 6/18 in the right eye and counting fingers close to face in the left. There were 1+ anterior chamber cells and 2+ vitreous cells in the right eye, and 2+ anterior chamber and vitreous cells in the left eye. There was no evidence of any mass lesion in the iris or fundus. She had undergone a Whipple′s procedure for periampullary adenocarcinoma 5 years ago. The histopathology report revealed a tumour restricted to the duodenal mucosa in the region of the Ampulla of Vater, with all resection limits free of malignancy [Figure - 1]. The adjacent pancreas and gall bladder were normal.

She was clinically diagnosed with Uveitis Masquerade Syndrome and was scheduled for a vitreous biopsy of the left eye. The patient refused any intervention and followed up four months later, with hand-motions vision in the right eye and counting fingers close to face in the left. There were 1+ anterior chamber cells with severe vitritis (vitreous cells 3+) in both eyes. A complete investigative work-up for pan-uveitis was done, and tests for tuberculosis, toxoplasmosis, syphilis and HIV were negative.

A diagnostic 3-port pars plana vitrectomy was done in the right eye. An undiluted vitreous sample (0.3ml) was collected in a sterile 1.0 ml syringe at the commencement of surgery. The fresh sample was immediately transported to the cytology laboratory and processed within 15 minutes. It was cytocentrifuged (ThermoShander Cytospin 3 ®, Cheshire, UK) at 1000 rpm for 5 minutes. The smear was then air-dried, stained with May-Grunwald-Giemsa stain and examined microscopically, but did not show any malignant cells. All microbiological investigations for bacteria and fungi were negative.

The ocular inflammation persisted in spite of treatment with systemic and topical corticosteroids. A diagnostic anterior chamber tap taken from the left eye revealed scattered neutrophils and no malignant cells. This eye meanwhile developed a significant cataract precluding any view of the posterior segment. The ocular inflammation still persisted with 1+ to 2+ anterior chamber cells and 2+ to 3+ vitreous cells in the right eye over the next 6 months in spite of oral and topical corticosteroid treatment. This prompted a repeat diagnostic vitrectomy. The vitreous sample was again processed for cytological examination as detailed above. Cytology of this specimen now revealed malignant cells suggestive of metastatic adenocarcinoma [Figure - 2].

A thorough postoperative systemic evaluation was conducted to ascertain any extraocular metastatic site. All biochemical investigations including blood films, renal and liver function tests were normal. A whole-body CT scan raised the suspicion of cerebral metastasis [Figure - 3], which was confirmed on MRI [Figure - 4]. The abdomen and chest were normal. There were no skeletal metastases on bone-scan. The patient received external beam radiation in both eyes (30 Gy over 10 days), and palliative chemotherapy.

On last follow-up 9 months following diagnosis, the patient had 1/60 vision in the right eye, and was scheduled for cataract surgery in the left eye.

  Discussion Top

Masquerade Syndromes constitute a small percentage of uveitis cases encountered in ophthalmic practice. Rothova et al2 surveyed 828 patients with uveitis and found 19 with underlying malignancies masquerading as uveitis; 16 of their patients had lymphoma or leukaemia, and only one patient had metastasis from a non-ocular solid tumour (lung). Anterior segment metastases are more likely to be misdiagnosed as uveitis, since the friable tumours shed malignant cells, and may present with cellular reaction, iris nodules, rubeosis iridis and elevated intraocular pressure.[1]

The neoplastic conditions commonly giving rise to intraocular malignant cells are intraocular reticulum cell sarcoma, central nervous system B-Cell lymphoma, leukaemia, uveal melanoma, retinoblastoma, and juvenile xanthogranuloma.[1] Although tumour involvement of the vitreous cavity is unusual with primary neoplasms, it poses a serious diagnostic challenge when it does occur. A literature search (Medline) revealed 16 cases of non-ocular solid tumours with ocular metastasis masquerading as uveitis, 9 of which were from the lung or breast. The other reported cancers[3]-[5] included ovarian choriocarcinoma, seminoma, adenocarcinoma tail of pancreas, neuroendocrine oat-cell tumour, non-Hodgkin′s lymphoma of jejunum and two cases of mucinous adenocarcinoma detected after enucleation, with unknown primary.

A clinical distinction between neoplastic and inflammatory cellular infiltration of the vitreous may be extremely difficult, necessitating cytological examination of intraocular fluid. Anterior chamber paracentesis is a useful diagnostic tool with less morbidity than a vitreous biopsy, though Woog et al6 have suggested that a 3+ cellular reaction is essential to obtain a useful cytology specimen.

A diagnostic pars plana vitrectomy (PPV) mainly helps differentiate inflammatory from neoplastic conditions.[7],[8] In our patient, a vitrectomy and aqueous tap did not demonstrate any tumour cells initially but a subsequent vitreous biopsy proved diagnostic.

This, to the best of our knowledge, is the first reported case of intraocular spread of metastatic periampullary duodenal adenocarcinoma masquerading as uveitis 5 years after successful tumour resection. This case highlights the importance of maintaining a high index of suspicion for metastasis in any elderly patient with uveitis. Investigations must be tailored towards identification of these tumour cells, so as to be able to institute appropriate palliative therapy as early as possible.

  References Top

Read RW, Zamir e, Rao NA. Newoplastic masquerade syndromes. Surv Ophthalmology 2002;47:81-124.  Back to cited text no. 1
Rothova A, Ooizman F, Kerkhoff F, Van der Lelij A, Lokhorst H, Uveitis masquerade syndromes. Ophthalmol 2001;108:386-99.  Back to cited text no. 2
Frank KW, Sugar HS, Shermal AL, Beckman T, Thomas S. Anterior chamber metastasis from an overian choriocarcinoma. Am J Ophthalmol 1979;87:778-82.  Back to cited text no. 3
Rotkis WM, Kulander BG, Chandler JW, Kaiser FS. Diagnosis of anterior chamber metastasis by serologic marker found during anterior chamber paracentesis. Am J Ophthalmol 1986;102:179-81.  Back to cited text no. 4
Barsky D. Unusual tumor of the iris. A rare initial clinical manifestation of metastatic adenocarcinoma of the tail of pancreas. Ann Ophthalmol 1978;10:1539-43.  Back to cited text no. 5
Woog JJ, Chess J, Alber DM, Berson FG, Raft J. Metastatic carcinoma of the iris simulating iridocyclitis. Br J Ophthalmol 1984;68:167-73.  Back to cited text no. 6
Verbraeken H. Diagnostic vitrectomy and chronic uveitis. Graefes Arch Clin Exp Ophthalmol 1996;234 Suppl:S2-7.  Back to cited text no. 7
Palexas GN, Green WR, Goldberg MF, Ding Y. Diagnostic pars plana vitrectomy: Report of a 21 year retrospective study. Trans Am Ophthalmol Soc 1995;93:281-14.  Back to cited text no. 8


  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]

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