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pathogenesis to be a mere allergic reaction. The mass in both eyes had identical histopathological fi ndings, but only the mass in the right eye followed a refractory course. This implies that the mass formation was not a secondary reac-tion to the chemical ocular surface injury that the patient suffered just before his initial visit. Thus, we speculate that the pathogenesis of this condition could be partly associated with the atopic dermatitis. Despite the recurrent course, surgical resection could provide short-term relief from the symptoms. Further investigations are needed to elucidate the nature of this condition and its treatment.

Keywords: eosinophilic, granulation tissue, ligneous conjunctivitis, pyogenic granuloma, vernal conjunctivitis

Yoshimichi Matsunaga, Yoshihiko Usui, Kenichiro Kasai, Yu Sakurai, and Hiroshi GotoDepartment of Ophthalmology, Tokyo Medical University, Tokyo, Japan

Received: October 21, 2008 / Accepted: April 17, 2009Correspondence to: Yoshimichi Matsunaga, Department of Ophthal-mology, Tokyo Medical University, 6-7-1 Nishi-shinjuku, Shinjuku, Tokyo 160-0023, Japane-mail: Yoshimichi.matsunaga@gmail.com

DOI 10.1007/s10384-009-0720-5

References

1. Parikh JG, Khurana RN, Lai MM, Rodriguez A, Rao NA. Keloid of the conjunctiva simulating a conjunctival malignancy. Br J Oph-thalmol 2007;91:1251–1252.

2. Patrice SJ, Wiss K, Mulliken JB. Pyogenic granuloma (lobular capil-lary hemangioma): a. Clinicopathologic study of 178 cases. Pediatr Dermatol 1991;8:267–276.

3. Schuster V, Seregard S. Ligneous conjunctivitis. Surv Ophthalmol 2003;48:369–388.

4. Buggage RR, Spraul CW, Wojno TH, Grossniklaus HE. Kimura disease of the orbit and ocular adnexa. Surv Ophthalmol 1999;44:79–91.

5. Jayamanne DG, Webber SK, Ridley RE, Cottrell DG, McCarthy JH. Angiolymphoid hyperplasia with eosinophilia (Kimura’s disease) of the conjunctiva. Br J Ophthalmol 1995;79:1053–1054.

Squamous Cell Carcinoma of the Conjunctiva Invading the Orbit in

a Non-Hodgkin Lymphoma

Non-Hodgkin lymphoma (NHL), which is a heterogeneous group of lymphoproliferative malignancies, appears to be associated with an increased risk of multiple other malig-nancies such as lung, brain, and skin cancers, and Hodgkin disease.1 Squamous cell carcinoma (SCC) is believed to be the most common second primary malignancy in patients with malignant lymphoma.2

To our knowledge, a few cases of SCC in NHL patients have been reported,2,3 but none of these involved surgery or were reported by ophthalmologists.

In this paper, we present a case of an NHL patient with newly developed SCC of the conjunctiva who was treated with ophthalmic surgery.

Case Report

In March 2005, a 78-year-old man presented in our depart-ment with an orbital mass in the left inferior fornix. He had been diagnosed in 2001 as having NHL (stage IIA according to the Ann Arbor classifi cation system; an angioimmuno-blastic T-cell lymphoma according to the World Health Organization classifi cation system) and since 2003 had been in complete remission after having undergone six cycles of chemotherapy [three cycles of CHOP (cyclophospha-mide, doxorubicin, vincristine, and prednisolone) after three cycles of CVP (cyclophosphamide, vincristine, and prednisolone)].

He had had no light perception in the left eye for a long time, but further evaluation was impossible because of an old corneal scar. A pinkish, fi rm, nontender, and un-movable mass was visible in the inferior fornix (Fig. 1A). Computed tomography (CT) showed a homogenous mass surrounding the inferior and medial aspects of the eyeball,

Figure 1A, B. A 78-year-old man presented with an orbital mass in the left inferior periorbital area. A A conjunctival mass was visible in the left inferior fornix. B A computed tomography scan showed a homogenous mass sur-rounding the inferior and medial aspects of the eyeball.

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but nothing was remarkable in other regions such as the paranasal sinuses or upper airway, which are common sites for head and neck tumors (Fig. 1B). Incisional biopsy of the underlying mass revealed moderately differentiated SCC of the conjunctiva invading the orbital cavity (Fig. 2A). Because orbital CT showed an increase in the size of the mass after the radiotherapy, we decided to perform orbital exenteration with radical mass excision in December 2005.

The surgery was successful, with no residual mass. The specimen showed that the SCC had invaded the anterior chamber, vitreal cavity, surrounding orbital tissue, and eyelid (Fig. 2B). The immunohistochemical report was positive for p53, Ki-67, CK7, and p63.

Unfortunately, multiple metastases were found in the patient’s forehead, cheek, and nasopharyngeal area 1 year after surgery. Despite further chemotherapy for SCC, he died the following year.

Comments

Several studies have shown an increased risk of multiple other malignancies in patients with NHL. Kyasa et al.1 reported a signifi cantly increased risk of skin cancer (includ-ing SCC) in patients with NHL. Moreover, the development of SCC has been reported after long-term remission of NHL.3

Many mechanisms have been postulated to explain the increased association of NHL and SCC, such as disease-related immunosuppression, immunosuppressive effects of the chemotherapy and radiation, genetic abnormality, viral infection, and ultraviolet radiation.4

In our case, the patient had been in complete remission for 2 years when the SCC occurred, and, whereas there was no evidence of immunosuppression at that time, he did have a son with SCC of the orbit. We therefore presumed that inherent susceptibility was a highly possible cause of the carcinoma. Thus far, many genetic lesions have been sug-gested to be associated with SCC development. The most prominent aberration is a mutation of the p53 suppressor gene, which was also found in our case.4

The prognosis of SCC in NHL is usually poor because of its high recurrence rate, reported to be seven times that in matched controls.5 Despite receiving the appropriate surgery, chemotherapy, and radiotherapy, the patient in our case died.

In conclusion, a newly developed orbital mass in a patient with known malignant lymphoma may not always indicate metastasis. Because of the increased risk of multiple other malignancies, the possibility of a second primary malig-nancy, especially SCC, should always be borne in mind.

Keywords: conjunctiva, lymphoma, multiple malignancies, orbit, squamous cell carcinoma

Soon-Il Kwon, Yun Suk Chung, and Yun-Jeong KimDepartment of Ophthalmology, Hallym University Sacred Heart Hospital, Anyang, Korea

Received: November 1, 2008 / Accepted: July 1, 2009Correspondence to: Yun-Jeong Kim, Department of Ophthalmology, Hallym University Sacred Heart Hospital, #896 Pyungchon-dong, Anyang, Gyeonggi-do 431-070, Koreae-mail: bonamd@paran.com

DOI 10.1007/s10384-009-0733-0

References

1. Kyasa MJ, Hazlett L, Parrish RS, Schichman SA, Zent CS. Veterans with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) have a markedly increased rate of second malignancy, which is the most common cause of death. Leuk Lymphoma 2004;45:507–513.

2. Watanabe N, Inohara H, Akahani S, Yamamoto Y, Moriwaki K, Kubo T. Synchronous squamous cell carcinoma and malignant lym-phoma in the head and neck region. Auris Nasus Larynx 2007;34:273–276.

3. Cheon HC, Lee DY, Park WC, Ahn HB. Ocular surface squamous neoplasia. J Korean Ophthalmol Soc 2006;47:1920–1928.

4. Boukamp P. Non-melanoma skin cancer: what drives tumor devel-opment and progression? Carcinogenesis 2005;26:1657–1667.

5. Mehrany K, Weenig RH, Lee KK, Pittelkow MR, Otley CC. High recurrence rates of squamous cell carcinoma after Mohs’ surgery in patients with chronic lymphocytic leukemia. Dermatol Surg 2005;55:639–642.

Figure 2. A Histopathology shows invasive, irregular masses composed of atypical squamous cells (H&E; bar = 100 μm). B The specimen after exenteration shows that a whitish mass had infi ltrated the surrounding orbital tissue and thickened the upper and lower eyelids by up to 1 cm.