Conjunctival Metastasis from Leiomyosarcoma of Anterior Chest Wall View PDF

^*Sonal P Yadav
Department Of Ophthalmic Plastic Surgery, Orbit And Ocular Oncology, PBMA HV Desai Eye Hospital, Maharashtra , India

^*Corresponding Author:

Sonal P Yadav
Department Of Ophthalmic Plastic Surgery, Orbit And Ocular Oncology, PBMA HV Desai Eye Hospital, Maharashtra , India
Email:sonal.yadav@hvdeh.org

Published on: 2020-12-29

Abstract

Leiomyosarcoma is the most common soft tissue sarcoma and is described as a malignant tumor of mesenchymal cells. It is rarely found as primary, secondary, or metastatic in origin in the eye and adnexa. To the best of our knowledge, a metastatic leiomyosarcoma involving the conjunctiva has not been reported in the literature. We report a case of a 35-years-old gentleman, with a prior history of leiomyosarcoma involving the anterior chest wall with pulmonary metastasis presenting to us with a rapidly increasing conjunctival mass involving his left eye. After a detailed clinical evaluation, the lesion was surgically excised. We discuss the clinical features and histopathology characteristics supported by immunohistochemistry analysis of this rare case of conjunctival metastasis from leiomyosarcoma with a review of metastatic tumors of the conjunctiva.

Keywords

Conjunctival Metastasis, Leiomyosarcoma, Anterior Chest Wall

Introduction

Metastatic tumor to conjunctiva is a rare entity and it may often reflect poor prognosis due to an advanced state of the primary malignancy. They constitute <1% of all conjunctival tumors [1]. Leiomyosarcoma is a malignant tumor of mesenchymal cells with smooth muscle differentiation. Primary leiomyosarcoma originating from the eye and adnexa has been reported from conjunctiva, uvea, and orbit [2-4]. Metastatic leiomyosarcoma is extremely rare and has been described in orbit, eyelid, and choroid in literature [5-8]. To the best of our knowledge, leiomyosarcoma metastatic to conjunctiva has not been reported previously. We describe a case of a 35-years-old gentleman who presented to us with a rapidly growing nodule on his bulbar conjunctiva with a history of biopsy-proven left-sided anterior chest wall leiomyosarcoma with a review of metastatic tumors of the conjunctiva. The patient’s consent was obtained for use of his clinical photographs for academic and research purposes. The case report adheres to the ethical principles outlined in the Declaration of Helsinki as amended in 2013.

Case Report

A 35-years-old gentleman presented to us with a rapidly increasing single reddish nodule on the nasal bulbar conjunctiva of the left eye noticed for 2 weeks. It measured11x10x6mm, showed superficial congestion with intense intrinsic vascularity and episcleral fixity (Figure 1).

His best-corrected visual acuity was 20/20, both eyes with an unremarkable posterior segment in the left eye. The right eye examination was within normal limits. There was no palpable lymphadenopathy on the either side. On review of prior records, he was a diagnosed (biopsy-proven) case of leiomyosarcoma of the anterior chest wall with metastasis to lungs 8 years back and was treated with surgery, radiation, and adjuvant chemotherapy elsewhere. He was in remission as per the treating oncosurgeon until his current presentation. His routine blood investigations along with HIV serology was found to be within normal limits.

On detailed evaluation, the suspicion of metastatic nature of the solitary conjunctival nodule was considered. The nature of the ailment, pros, and cons of surgical treatment was discussed with the patient. He underwent wide excision of the conjunctival nodule with double freeze-thaw cryotherapy to the margins followed by fibrin glue-assisted surface reconstruction with amniotic membrane. Intra-operatively, the nodule was found to be adherent to the underlying sclera. A controlled lamellar sclerectomy was performed to excise maximum tumor residue. On histopathology evaluation (H & E), the tumor was composed of spindle and pleomorphic cells with markedly atypical nuclei and brisk mitotic activity. (Figure 2A and 2B) Immunohistochemical (IHC) analysis revealed that the tumor cells were strongly positive diffusely for smooth muscle actin (SMA) (Figure 2C) and focally positive for Desmin (Figure 2D). Cytokeratin was absent. The features were consistent with metastatic leiomyosarcoma involving the conjunctiva.

The wound healing was satisfactory at the time of 6 weeks review with minimal residual scarring. (Figure 3A and B) As the base of the excised lesion was involved by the tumor, he was advised to undergo episcleral plaque brachytherapy to the tumor base along with systemic metastatic evaluation (Whole body PET CT scan) to look for progression at the primary site and/or additional distant metastasis. However, the patient has been non-compliant for further investigations and management. On the last contact, 9 months post-surgery, he is alive and stable.

Discussion

Metastatic tumors to conjunctiva present as rapidly growing, fleshy, pink, vascularised lesion often non-adherent to the underlying sclera [9]. The clinical presentation of the current case was as per the previous description except we encountered metastatic conjunctival nodule adherent to and involving underlying sclera. In absence of a prior documented history of anterior chest wall leiomyosarcoma, the clinical differential diagnosis of the solitary conjunctival nodule in a young male would have included nodular episcleritis or nodular scleritis, foreign body granuloma, lymphoproliferative disease, or occasionally a mucoepidermoid variant of squamous cell carcinoma, myxoid, myogenic or histiocytic tumor [10]. The previous history and availability of histopathology diagnosis of primary malignancy along with immunohistochemistry aided histopathology evaluation of the present lesion helped to secure a rare diagnosis of metastatic conjunctival tumor from a leiomyosarcoma. On an extensive literature search on the PubMed database, we could not find a prior published case of metastatic conjunctival tumor from leiomyosarcoma making this the first reported case.

Metastasis to conjunctiva is notably rare. On PubMed search with keywords “conjunctiva/val” and “metastasis” in the English language, we found only 3 large series and few individual case reports describing metastasis to conjunctiva [1,9,11-33]. (Summarised in Table 1). The most common primary malignancy site was breast 14 cases followed by cutaneous melanoma 13 cases and lung 9 cases. Other reported primary malignancy sites include the larynx, gastric, renal, visceral Kaposi, parotid gland, utero-cervix, and vagina. Conjunctival metastasis was found on bulbar conjunctiva in the majority of reported cases followed by palpebral, tarsal, and forniceal sites. In most of the reported cases, the conjunctival metastasis was accompanied by rest of ocular structure involvement and/or wide-spread distant organ metastasis. Excision for conjunctival site coupled with definitive or palliative systemic chemotherapy and/or radiotherapy was employed for management [1,9,11-33] (Table 1).

Table 1: Review of published cases of conjunctival metastasis (1,9,11-33).

No	Author(s), year	No of cases	Primary malignancy	Conjunctival metastasis site	Treatment
1	Grossniklaus, et al. (1987) [11]	1	Not specified	Not specified	Not specified
2	Jakobiec, et al. (1989) [12]	5	Cutaneous melanoma	Bulbar (2) Fomical (1)	Excision + treatment of primary malignancy
				Plical-caruncular (1)
				Palpebral (1)
3	Ortiz, et al. (1995) [13]	1	Uterine cervical carcinoma	Bulbar	Excision + systemic chemotherapy and surgery for primary site
4	Kiratli, et al. (1996) [9]	10	Breast (4)	Bulbar (6)	EBRT (5)
			Lung (2)	Palpebral (2)	Excision with EBRT (1)
			Cutaneous melanoma (2)	Limbal (1)	Enucleation with EBRT (1)
			Larynx (1)	Fornix (1)	Excision (1)
			Unknown (1)		Chemo (1)
					None (1)
5	Kwapiszeski, et al. (1997) [14]	1	Cutaneous melanoma	Bulbar	Excision + systemic chemotherapy for primary malignancy
6	Shields, et al. (1998) [15]	1	Lung	Bulbar	Enucleation (multiple ocular site involvement)
7	Ziakas, et al. (2000) [16]	1	Cutaneous melanoma	Palpebral	Palliative therapy
8	Alwitry, et al. (2001) [17]	1	Lung	Bulbar	Palliative therapy
9	Tokuyama, et al. (2002) [18]	1	Gastric cancer (signet ring carcinoma)	Bulbar	Excision + Primary site surgery
10	Shields, et al. (2004) [19]	1	Cutaneous melanoma	Bulbar	Excision+ systemic treatment for primary and other metastatic sites
11	Shields, et al. (2004) (1)	13	Breast (5)	Bulbar (13)	Not specified
			Lung (3)
			Cutaneous melanoma (1)
			Larynx (1)
			Carcinoid tumor (1)
			Unknown (1)
12	Pompeu, et al. (2005) [20]	1	Renal cell carcinoma	Tarsal	Excision and primary site surgery
13	Skalicky, et al. (2007) [21]	1	Breast	Bulbar	Palliative systemic therapy for primary and other metastatic sites
14	Cano, et al. (2011) [22]	1	Visceral Kaposi sarcoma	Palpebral	Excision
15	Radovanovi?, et al. (2013) [23]	1	Breast	Bulbar	Excision + palliative radiotherapy + hormonal therapy to primary and other metastasis sites
16	Chung, et al. (2004) [24]	1	Lung (Pleural mesothelioma)	Bulbar	Palliative therapy
17	Park, et al. (2014) [25]	1	Breast	Bulbar	Excision + systemic chemotherapy
18	Chew, et al. (2014) [26]	1	Lung	Palpebral	Excision + radiotherapy to primary and other metastatic sites
19	Sahin, et al. (2014) [27]	1	Lung	Bulbar	Not specified
20	Carey, et al. (2014) [28]	1	Cutaneous melanoma	Bulbar	Excision and systemic chemotherapy for primary site
21	Diltoer, et al. (2015) [29	1	Breast	Bulbar	Not specified
22	Yadav, et al. (2017) [30]	1	Mucoepidermoid carcinoma of parotid gland	Bulbar	Orbital exenteration
23	Sanchez, et al. (2017) [31]	1	Breast	Bulbar	Palliative therapy
23	Brouwer, et al. (2018) [32]	1	Cutaneous melanoma	Fornix	Excision + systemic chemotherapy
24	Phelps, et al. (2018) [33]	1	Vaginal melanoma	Tarsal	Systemic chemotherapy, Radiation to brain (another metastatic site)
25	Our case, 2020	1	Leiomyosarcoma of Anterior chest wall	Bulbar	Excision + Episcleral plaque brachytherapy (advised)

Leiomyosarcoma is the most common soft tissue sarcoma. It can arise from multiple sites including soft tissue (48%), skin (14%), gastrointestinal tract (7%), uterus (7%), and retroperitoneum (7%) [34]. Primary leiomyosarcoma of the chest wall is rare and reportedly accounts for approximately 1-4% of primary soft tissue sarcomas of the chest wall [35]. Distant metastasis from chest wall leiomyosarcoma can occur in the liver or lung via a hematogenous route [36]. Metastasis from leiomyosarcoma to the eye and periorbita are extremely rare. Conjunctival metastases like in our case are most likely to occur via hematogenous spread as well. Our patient underwent complete surgical excision and was advised to undergo episcleral plaque brachytherapy for the involvement of base sclera. The status of other distant organ metastasis was unknown as he was non-compliant for further investigations and management. The wound had healed with minimal scarring without any recurrence at 6 weeks and on the last telephonic contact at 9 months he was alive and stable.

In summary, we report a rare case of anterior chest wall leiomyosarcoma metastatic to the conjunctiva. A high index of suspicion regarding possible metastasis should be considered when encountered a solitary conjunctival nodule in a patient with known primary malignancy. Histopathology and immunohistochemistry are essential tools for accurate diagnosis and that help in guiding the optimum management in such rare cases.