Conjunctival leiomyosarcoma

Letters to the Editor imaging modalities would have been helpful; unfortunately this patient did not have prior imaging to allow for comparison. In conclusion, we believe PET/CT is a viable investigative option to exclude low-grade chronic orbital implant infection. To the best of the authors’ knowledge, the first documented use of PET/CT in the diagnosis of infected orbital porous implant.

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WengOnn Chan MBChB,1 Eugenie Poh FRCSEd(Ophth),1 Dylan Bartholomeusz MD FRACP2 and Dinesh Selva FRANZCO1 1 South Australian Institute of Ophthalmology, and 2 Department of Nuclear Medicine, PET & Bone Densitometry, Royal Adelaide Hospital, Adelaide, South Australia, Australia Received 29 December 2010; accepted 7 January 2011.

REFERENCES 1. Jordan DR, Brownstein S, Faraji H. Clinicopathologic analysis of 15 explanted hydroxyapatite implants. Ophthal Plast Reconstr Surg 2004; 20: 285–90. 2. Karslog˘lu S, Serin D, Sims¸ek I, Ziylan S. Implant infection in porous orbital implants. Ophthal Plast Reconstr Surg 2006; 22: 461–6. 3. Kristinsson JK, Sigurdsson H, Sigfússon A, Gudmundsson S, Agnarsson BA. Detection of orbital implant infection with technetium 99m-labeled leukocytes. Ophthal Plast Reconstr Surg 1997; 13: 256–8. 4. Kumar R, Basu S, Torigian D, Anand V, Zhuang H, Alavi A. Role of modern imaging techniques for diagnosis of infection in the era of 18F-fluorodeoxyglucose positron emission tomography. Clin Microbiol Rev 2008; 21: 209–24.

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Choroidal metastasis from primary pulmonary leiomyosarcoma We report a case of primary pulmonary leiomyosarcoma metastatic to the choroid, which to our knowledge has not previously been described. A 22-year-old woman underwent right pneumonectomy and received adjuvant chemotherapy (doxorubicin and ifosfamide) for a high-grade, primary pulmonary leiomyosarcoma. Seven months later, she was referred to our service with a choroidal tumour in the left eye. On examination, the visual acuity with the left eye was hand movements. Nasal sentinel vessels were noted and a large, amelanotic tumour was seen arising from the nasal choroid, associated with an inferior serous retinal detachment (Fig. 1a). The right eye was healthy, with a visual acuity of 6/5 in part. B-scan ultrasonography showed a large, mushroomshaped tumour arising from the nasal, peripheral choroid, measuring 16.3 mm ¥ 17.7 mm, with a thickness of

Figure 1. Clinical features of choroidal metastatic lesion. (a) Colour photograph showing sentinel vessels and a large, amelanotic, posterior segment mass; (b) B-scan ultrasound showing a mushroom-shaped mass arising from the peripheral choroid with an associated retinal detachment; (c) A-scan showing a low internal reflectivity and irregular pattern.

© 2011 The Authors Clinical and Experimental Ophthalmology © 2011 Royal Australian and New Zealand College of Ophthalmologists

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Figure 2. Excision of mass lesion. (a) Intraoperative photograph showing trans-scleral resection of the tumour; (b) low-power magnification of the resection specimen (HE, ¥2 objective); (c) atypical spindle cells with scattered inflammatory cells, blasts and mitoses (see arrow, inset) (HE, ¥40 objective); (d) immunoreactivity for desmin (a smooth muscle marker) (APAAP, ¥40 objective).

12.9 mm (Fig. 1b). Standardized A-scan showed low internal reflectivity and mild vascularity. Irregularity in the A-scan pattern was more than that typically seen in choroidal melanoma (Fig. 1c). Trans-retinal tumour biopsy showed features consistent with a metastasis from a pulmonary leiomyosarcoma. The patient was therefore referred for external beam radiotherapy and received a dose of 46 Gray in 23 fractions. At review 17 days later, the absence of tumour shrinkage indicated that the lesion had not responded to radiotherapy. We therefore proceeded to trans-scleral local

resection of the tumour under hypotensive anaesthesia (Fig. 2a). Postoperatively the patient developed a rhegmatogenous retinal detachment and underwent surgical repair with lensectomy, which was successful. Six months following tumour resection the corrected visual acuity with silicone oil in situ was perception of hand movements. The retina was flat, and there was no local visible tumour recurrence. Histopathological examination of the resected tumour showed atypical spindle cells with moderate cytoplasm

© 2011 The Authors Clinical and Experimental Ophthalmology © 2011 Royal Australian and New Zealand College of Ophthalmologists

Letters to the Editor and numerous lymphocytes, plasma cells, eosinophilic granulocytes and macrophages. Scattered multinucleate cells and atypical mitoses were also present. The spindle cells were immunoreactive for smooth muscle markers and negative for melanocytic markers (MelanA) (Fig. 2b–d). The Ki-67 growth fraction was low, c. 15%. These features were identical to those of the histological sections of the primary pulmonary tumour, which were retrieved and reviewed. Primary uveal leiomyosarcoma has been reported previously.1,2 To our knowledge, however, this is the first case in the English literature of pulmonary leiomyosarcoma metastatic to the choroid. The vast majority of uveal metastases originate from carcinomas;3 metastases arising from sarcomas are rare, with most being from the urogenital tract. In a series of 838 choroidal metastases, Shields et al. noted that only two lesions were mushroom-shaped, commenting that this morphology is highly suggestive of a choroidal melanoma.3 However, our impression was that the lesion was not echographically typical of choroidal melanoma because of the higher degree of irregularity seen on standardized A-scan. In view of the systemic history, secondary choroidal leiomyosarcoma was considered the most likely diagnosis. We performed biopsy to direct our management. Histological confirmation of metastatic leiomyosarcoma led us to administer radiotherapy, which failed. It is known that leiomyosarcoma is relatively radioresistant,4 certainly less responsive to radiotherapy than the more common epithelial malignancies that metastasize to the choroid. Trans-scleral local resection achieved local tumour control in this case. In retrospect, primary resection would probably have been the most appropriate management here. This case report is a reminder that management of ocular metastasis should be influenced by the diagnosis of the primary tumour and the known responsiveness of this tumour to radiotherapy and chemotherapy.

Matthew F Anderson MBChB,1 Sarah E Coupland PhD,2 Donald Bissett MD,3 Hatem Atta MBChB4 and Bertil E Damato PhD1 1 Liverpool Ocular Oncology Centre, St. Paul’s Eye Clinic, Royal Liverpool University Hospital, 2Department of Pathology, University of Liverpool, Liverpool, 3Aberdeen Royal Infirmary, and 4NHS Grampian and University of Aberdeen, Aberdeen, UK Received 28 August 2010; accepted 26 January 2011.

REFERENCES 1. Cajaiba MM, Chojniak MM, Cunha IW. Unusual primary ocular neoplasm in a child: leiomyosarcoma of the ciliary body. Pediatr Dev Pathol 2008; 11: 479–81. 2. Park S, Kim H, Chin H, Tae K, Han J. Mesectodermal leiomyosarcoma of the ciliary body. Am J Neuroradiol 2003; 24: 1765–8.

707 3. Shields CL, Shields JA, Gross NE, Schwartz GP, Lally SE. Survey of 520 eyes with uveal metastases. Ophthalmology 1997; 104: 1265–76. 4. Ferrari A, Collini P, Casanova M, Meazza C, Podda M, Mazza EA. Response to chemotherapy in a child with primary bronchopulmonary leiomyosarcoma. Med Pediatr Oncol 2002; 39: 55–7.

Microperimetry evidence of functional improvement after vitrectomy for optic disc pit-related intraretinal fluid without serous detachment Congenital optic disc pit (ODP) occurs at a rate of 1:11 000 and causes macular pathology in 25–75%.1 Spectral domain optical coherence tomography (SDOCT) studies demonstrated that fluid arising from an ODP can go directly to the sub-retinal space and the various layers of the neurosensory retina including the sub-internal limiting membrane (sub-ILM) space.2 Although it is known that sub-retinal fluid leads to visual loss, the impact of intraretinal fluid – so-called retinoschisis,3 on retinal function has not been studied in detail. We report the microperimetry and SDOCT findings from a case of ODP with extensive intraretinal fluid without serous retinal detachment, pre- and post-vitrectomy, to correlate functional improvement with anatomical recovery. A 31-year-old Caucasian woman with ODP presented with gradual visual loss in her right eye for 6 years. Serial visual field tests over 5 years demonstrated enlarging superior arcuate defect (Fig. 1a). Over the same period, an inferotemporal scotoma connected to the physiological blind spot developed and subsequently extended to the inferonasal quadrant. Her best-corrected visual acuities (VA) were 6/12 R and 6/6 L. Fundus examination revealed an ODP associated with adjacent neuroretinal rim notching and corresponding nerve fibre layer (NFL) thinning. A 20-disc area of para- and extra-macular retinal thickening was noted, extending superiorly from the disc margin and along the superotemporal arcade. There was no sub-retinal fluid. The left eye was normal. The patient declined surgery and agreed to be monitored by serial microperimetry (Nidek MP1 microperimeter, Nidek Technologies, Padova, Italy) and SDOCT (Topcon 3D-OCT 1000 and Spectralis OCT). Over the subsequent 10 months, her VA declined from 6/12 to 6/36 and microperimetry showed progressive paracentral retinal sensitivity loss (Fig. 1c). SDOCT demonstrated (i) intraretinal fluid at the level of Henle’s fibre layer (HFL) extending from the temporal disc margin to involve the entire macular region and (ii) intraretinal fluid at the level of inner plexiform layer (IPL) and NFL

© 2011 The Authors Clinical and Experimental Ophthalmology © 2011 Royal Australian and New Zealand College of Ophthalmologists