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Clinicopathologic Reports, Case Reports, and Small Case Series |

Report of a Conjunctival Myxoma Case and Review of the Literature FREE

Hakan Demirci, MD; Carol L. Shields, MD; Ralph C. Eagle Jr, MD; Jerry A. Shields, MD
[+] Author Affiliations

Section Editor: W. Richard Green,, MD

More Author Information
Arch Ophthalmol. 2006;124(5):735-738. doi:10.1001/archopht.124.5.735.
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Published online

Myxoma is a benign soft tissue tumor that presumably derives from primitive mesenchyme. It is characterized histopathologically by abundant mucoid material, a loose meshwork of reticulin fibers, and relatively small numbers of spindle- and stellate-shaped cells.1 The stromal matrix is rich in glucosaminoglycans and hyaluronic acid, and it is sparse in collagen and vascular structures.1 Myxoma can appear as localized disease or as a component of Carney complex, which includes cutaneous and cardiac myxomas, multiple pigmented lesions, and endocrine overactivity, leading to significant morbidity and mortality.2 Myxoma is the most common primary cardiac tumor. This tumor can arise in the skeletal muscle, bone, skin, genitourinary system, gastrointestinal tract, and nasal sinuses.3 It can rarely involve ocular structures and has been recognized in the eyelid, orbit, and conjunctiva.46

In a review of 2455 conjunctival lesions submitted to an ophthalmic pathology laboratory, only 4 patients (0.002%) were found to have conjunctival myxoma.4 In a clinical review of 1643 patients with conjunctival lesions, myxoma was found in 1 case (<0.001%).5 Because of its rarity, conjunctival myxoma can simulate other conjunctival tumors, such as amelanotic nevus, amelanotic melanoma, squamous cell carcinoma, lipoma, and cyst.6 We describe a 31-year-old African American man who had a yellow, translucent subconjunctival mass that proved to be a conjunctival myxoma on histopathological examination.

A 31-year-old African American man had a 2-year history of conjunctival swelling in his left eye. The lesion was diagnosed as a conjunctival cyst, and drainage was attempted. Eighteen months later, the swelling worsened and a solid conjunctival mass was noted, prompting referral to the Oncology Service, Wills Eye Hospital, Philadelphia, Pa. The patient had no history of ocular trauma and was otherwise healthy.

The visual acuity was 20/20 OU. The right eye was normal. External examination of the left eye disclosed a well-circumscribed, yellow-pink, translucent mass on the nasal bulbar conjunctiva (Figure 1). The mass had 2 parts, including a solid basal part measuring 12 × 11 × 6 mm and a cystic apical part measuring 5 × 5 × 2 mm. The clinical differential diagnosis included amelanotic nevus, amelanotic melanoma, myxoma, fibrous histiocytoma, and lipoma. The lesion was excised using the previously described no-touch technique.7

Place holder to copy figure label and caption
Figure 1

Anterior segment of the left eye showing a well-circumscribed, yellow-pink, translucent mass with a solid basal part and a cystic apical part on the nasal bulbar conjunctiva.

Graphic Jump Location

Histopathological examination revealed a lesion composed of loose, myxoid areas that alternated with areas of spindle and stellate cells, which was consistent with benign conjunctival myxoma (Figure 2). Both cellular groups had moderately large hyperchromatic and slightly pleomorphic nuclei and were embedded in a loose mucoid stroma. The stroma contained reticulin fibers, sparse small blood vessels, and collagen fibers (Figure 2). Some of the tumor cells had clear, circular, intranuclear inclusions, and some contained clear cytoplasmic vacuoles. Numerous mast cells were scattered throughout the myxoid stroma (Figure 3).

Place holder to copy figure label and caption
Figure 2

Conjunctival myxoma. The hypocellular stroma contains delicate collagen fibers (hematoxylin-eosin, original magnification ×50) (A) and abundant mucoid material confirmed by intense positive staining with Alcian blue (original magnification ×20) (B).

Graphic Jump Location
Place holder to copy figure label and caption
Figure 3

Hypocellular myxoma contains multinucleated spindle cells with intranuclear vacuoles. Arrow indicates mast cell (hematoxylin-eosin, original magnification ×250).

Graphic Jump Location

Systemic evaluations of the patient for cardiac, endocrine, and cutaneous abnormalities including echocardiography and levels of thyroid-stimulating hormone, growth hormone, and adrenocorticotropic hormone were negative. After 11 years of follow-up, the patient remained healthy without recurrence of the conjunctival mass or evidence of a systemic abnormality.

Myxoma rarely occurs in the conjunctiva. To our knowledge, only 22 well-documented cases of conjunctival myxoma have been published in the English literature (Table).821 The tumor usually occurs in adults and is uncommon in children. The mean age of the 22 published cases was 48 years (median age, 50 years; age range, 18-80 years), and 2 patients (9%) were younger than 20 years.821 Both sexes were affected equally.821 Similarly, a review of 58 patients who all had soft tissue myxomas revealed a mean age of 55 years, with only 1 patient (2%) younger than 20 years.3

Table Graphic Jump LocationTable Clinical Findings in 22 Published Cases of Conjunctival Myxoma*

Conjunctival myxoma is typically seen as a well-circumscribed, yellow-pink, translucent, cystic and/or solid mass.821 Of the 22 reported cases, the conjunctival mass was described as well-circumscribed in 20 cases (91%) and diffuse in 2 cases (9%).821 The color of the conjunctival mass, when mentioned, was yellow in 9 cases (50%), pink in 8 cases (44%), and white in 1 case (6%).821 The conjunctival lesion was translucent in 14 cases (64%) and solid in 8 cases (36%).821

Conjunctival myxoma has characteristic histopathological features.11,13,14 It consists of scattered, relatively small numbers of stellate- and spindle-shaped cells disposed in a mucinous matrix with delicate reticulin fibers, sparse vascular structures, and mature collagen fibers.11,13,14 The mucinous matrix is composed predominantly of hyaluronic acid, with a lesser amount of chondroitin sulfate. Ultrastructurally, myxoma cells have intracytoplasmic vacuoles and cytoplasmic filaments arranged in loose, wavy bundles.11,13,14 These vacuoles are consisted with dilated rough endoplasmic reticulum. It seems that the mucoid matrix and extracellular fibrils are produced by myxoma cells. There are also intranuclear vacuoles that are consistent with invaginations of markedly folded nuclear membrane. Immunohistochemical studies13,14,17,21 showed myxoma cells were immunoreactive for vimentin and α smooth muscle actin but negative for S-100 protein, desmin, and myoglobulin.

Conjunctival myxoma differs clinically and histopathologically from myxomas of extraocular soft tissues in some aspects. Unlike extraocular soft tissue myxomas, conjunctival myxoma often contains cyst-like cavities. The latter were noted in 9 of the 22 published cases.821 In our case, the patient was diagnosed as having a conjunctival cyst, and drainage was attempted before referral to our service. Additionally, on histopathological examination, mast cells can be observed in conjunctival myxoma.11,13,14

The differential diagnosis of conjunctival myxoma includes nevus, amelanotic melanoma, fibrous histiocytoma, cyst, lymphangioma, myxoid neurofibroma, spindle cell lipoma, rhabdomyosarcoma, and myxoid liposarcoma.6 Unlike conjunctival amelanotic nevus and melanoma, myxoma does not have prominent intrinsic vascularity or pigmentation.6 Myxoid neurofibroma can have a similar clinical picture. It contains cells with spindle-shaped nuclei with thick, wavy bundles of collagen and can be associated with systemic neurofibromatosis.11 Both fibrous histiocytoma and myxoma stain positively with Alcian blue, which is abolished by pretreatment with hyaluronidase. Unlike fibrous histiocytoma, myxoma does not show vascularity or significant nuclear pleomorphism.1

The preferred management of conjunctival myxoma is complete excisional biopsy. Of the 22 published cases with conjunctival myxoma, no recurrence or malignant transformation after excisional biopsy of the lesion was observed.821 In a review of 58 patients with myxomas of the soft tissue, only 2 (3%) had recurrence after 8 to 10 months following excision. These 2 patients underwent reexcision, and both were free of recurrent tumor after a mean of 5 years. Recurrence of myxoma can be related to inadequate excision, multicentricity of the tumor, the presence of genetic predisposition, or malignant transformation. In a review of 107 cardiac myxomas submitted to a pathology laboratory, atypical cells simulating malignancy were observed in only 3%.22

Myxomas can be a component of Carney complex, Mazabraud syndrome (bone fibrous dysplasia and intramuscular myxoma), and McCune-Albright syndrome (café au lait spots and multinodular goiter). Carney complex is an autosomal dominant syndrome that includes 2 or more of the following conditions: multiple foci of mucocutaneous pigmentation (face, trunk, lips, conjunctiva), cardiac and extracardiac (skin, breast) myxomas, endocrine overactivity (including Cushing syndrome), pituitary adenoma, unusual testicular tumors, and psammomatous melanotic schwannoma.23 Patients with myxoma, mucocutaneous pigmentation, and/or a family history of Carney complex should undergo a systemic evaluation including echocardiography, computed tomography or magnetic resonance imaging of the body, testicular ultrasound, analysis of the thyroid-stimulating hormone level, adrenocorticotropic hormone level, and growth hormone level, and genetic analysis (PRKAR1A gene at chromosome 17q2).23 Ophthalmic manifestations of Carney complex include facial and eyelid lentigines, pigmentation on the caruncle or conjunctival semilunar fold, and eyelid myxoma.23,24 Kennedy et al15 described a patient with Carney complex who had a myxoma of the palpebral conjunctiva and an additional lesion on his eyelid margin. None of the 22 published cases of conjunctival myxoma in the literature were associated with Mazabraud or McCune-Albright syndromes.821

In summary, conjunctival myxoma can appear as a well-circumscribed, translucent, yellow-pink conjunctival mass in middle-aged patients. Management is generally complete surgical resection.

Correspondence: Dr C. L. Shields, Oncology Service, Wills Eye Hospital, 840 Walnut St, Philadelphia, PA 19107 (carol.shields@shieldsoncology.com).

Financial Disclosure: None.

Funding/Support: This work was supported by the Macula Foundation, New York, NY (Dr C. L. Shields), the Eye Tumor Research Foundation, Philadelphia, Pa (Dr C. L. Shields), the Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Hospital, Philadelphia (Dr Eagle), and the Paul Kayser International Award of Merit in Retina Research, Houston, Tex (Dr J. A. Shields).

Weiss  SWGoldblum  JRFranz  M Benign soft tissue tumors of uncertain type. In:Enzinger  FMWeiss  SWeds. Soft Tissue Tumors.  St Louis, Mo Mosby2001;1045- 1053
Carney  JAGordon  HCarpenter  PCShenoy  BVGo  VL The complex of myxomas, spotty pigmentation and endocrine overactivity. Medicine (Baltimore) 1985;64270- 283
PubMed Link to Article
Ireland  DCRSoule  EHIvins  JC Myxoma of somatic soft tissues: a report of 58 patients, 3 with multiple tumors and fibrous dysplasia of bone. Mayo Clin Proc 1973;48401- 410
PubMed
Grossniklaus  HEGreen  WRLuckenbach  MChan  CC Conjunctival lesions in adults: a clinical and histopathologic review. Cornea 1987;678- 116
PubMed Link to Article
Shields  CLDemirci  HKaratza  ECShields  JA Clinical survey of 1643 melanocytic and nonmelanocytic conjunctival tumors. Ophthalmology 2004;1111747- 1754
PubMed Link to Article
Shields  JAShields  CL Fibrous, neural, xanthomatous and myxomatous tumors. In:Shields  JAShields  CLeds. Atlas of Eyelid and Conjunctival Tumors.  Philadelphia, Pa Lippincott Williams & Wilkins1999;277- 288
Shields  JAShields  CLDe Potter  P Surgical approach to conjunctival tumors: the 1994 Lynn B. McMahan Lecture. Arch Ophthalmol 1997;115808- 815
PubMed Link to Article
Ffooks  OO Myxoma of the conjunctiva. Br J Ophthalmol 1962;46374- 377
Link to Article
Doughman  DJWenk  RE Epibulbar myxoma. Am J Ophthalmol 1970;69483- 485
PubMed
Stafford  WR Conjunctival myxoma. Arch Ophthalmol 1971;85443- 444
PubMed Link to Article
Patrinely  JRGreen  WR Conjunctival myxoma: a clinicopathologic study of four cases and a review of the literature. Arch Ophthalmol 1983;1011416- 1420
PubMed Link to Article
Mottow-Lippa  LTso  MOMSugar  J Conjunctival myxoma: a clinicopathologic study. Ophthalmology 1983;901452- 1458
PubMed Link to Article
Pe’er  JIlsar  MHidayat  A Conjunctival myxoma: a case report. Br J Ophthalmol 1984;68618- 622
PubMed Link to Article
Pe’er  JHidayat  A Myxomas of the conjunctiva. Am J Ophthalmol 1986;10280- 86
PubMed
Kennedy  RHFlanagan  JCEagle  RC  JrCarney  JA The Carney complex with ocular signs suggestive of cardiac myxoma. Am J Ophthalmol 1991;111699- 702
PubMed
Horie  YIkawa  SNagata  MTamai  A Myxoma of the conjunctiva: a case report and review of the literature. Jpn J Ophthalmol 1995;3977- 82
PubMed
Ramaesh  KWharton  SBDhillon  B Conjunctival myxoma, Zollinger-Ellison syndrome and abnormal thickening of the inter-atrial septum: a case report and review of the literature. Eye 2001;15309- 312
PubMed Link to Article
Maucione  L Di una rara forma di tumore epibulbare (Mixoma puro) suttocongiuntivale. Arch Ottamol 1914;21300- 306
Majewska-Cembrynska  AMajewski  H Sluzak nagalkowy. Klin Oczna 1973;43827- 830
PubMed
El-Rifai  MGhaly  AF Myxoma of the conjunctiva. Bull Ophthalmol Soc Egypt 1975;6831- 35
PubMed
Konari  KSuzuki  JOyachi  H Ocular pain as the initial sign of conjunctival myxoma. Rinsho Ganka 1993;471361- 1364
Burke  APVirmani  R Cardiac myxoma: a clinicopathologic study. Am J Clin Pathol 1993;100671- 680
PubMed
Carney  JA Carney complex: the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas. Semin Dermatol 1995;1490- 98
PubMed Link to Article
Kennedy  RHWaller  RRCarney  JA Ocular pigmented spots and eyelid myxomas. Am J Ophthalmol 1987;104533- 538
PubMed

Figures

Place holder to copy figure label and caption
Figure 1

Anterior segment of the left eye showing a well-circumscribed, yellow-pink, translucent mass with a solid basal part and a cystic apical part on the nasal bulbar conjunctiva.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2

Conjunctival myxoma. The hypocellular stroma contains delicate collagen fibers (hematoxylin-eosin, original magnification ×50) (A) and abundant mucoid material confirmed by intense positive staining with Alcian blue (original magnification ×20) (B).

Graphic Jump Location
Place holder to copy figure label and caption
Figure 3

Hypocellular myxoma contains multinucleated spindle cells with intranuclear vacuoles. Arrow indicates mast cell (hematoxylin-eosin, original magnification ×250).

Graphic Jump Location

Tables

Table Graphic Jump LocationTable Clinical Findings in 22 Published Cases of Conjunctival Myxoma*

References

Weiss  SWGoldblum  JRFranz  M Benign soft tissue tumors of uncertain type. In:Enzinger  FMWeiss  SWeds. Soft Tissue Tumors.  St Louis, Mo Mosby2001;1045- 1053
Carney  JAGordon  HCarpenter  PCShenoy  BVGo  VL The complex of myxomas, spotty pigmentation and endocrine overactivity. Medicine (Baltimore) 1985;64270- 283
PubMed Link to Article
Ireland  DCRSoule  EHIvins  JC Myxoma of somatic soft tissues: a report of 58 patients, 3 with multiple tumors and fibrous dysplasia of bone. Mayo Clin Proc 1973;48401- 410
PubMed
Grossniklaus  HEGreen  WRLuckenbach  MChan  CC Conjunctival lesions in adults: a clinical and histopathologic review. Cornea 1987;678- 116
PubMed Link to Article
Shields  CLDemirci  HKaratza  ECShields  JA Clinical survey of 1643 melanocytic and nonmelanocytic conjunctival tumors. Ophthalmology 2004;1111747- 1754
PubMed Link to Article
Shields  JAShields  CL Fibrous, neural, xanthomatous and myxomatous tumors. In:Shields  JAShields  CLeds. Atlas of Eyelid and Conjunctival Tumors.  Philadelphia, Pa Lippincott Williams & Wilkins1999;277- 288
Shields  JAShields  CLDe Potter  P Surgical approach to conjunctival tumors: the 1994 Lynn B. McMahan Lecture. Arch Ophthalmol 1997;115808- 815
PubMed Link to Article
Ffooks  OO Myxoma of the conjunctiva. Br J Ophthalmol 1962;46374- 377
Link to Article
Doughman  DJWenk  RE Epibulbar myxoma. Am J Ophthalmol 1970;69483- 485
PubMed
Stafford  WR Conjunctival myxoma. Arch Ophthalmol 1971;85443- 444
PubMed Link to Article
Patrinely  JRGreen  WR Conjunctival myxoma: a clinicopathologic study of four cases and a review of the literature. Arch Ophthalmol 1983;1011416- 1420
PubMed Link to Article
Mottow-Lippa  LTso  MOMSugar  J Conjunctival myxoma: a clinicopathologic study. Ophthalmology 1983;901452- 1458
PubMed Link to Article
Pe’er  JIlsar  MHidayat  A Conjunctival myxoma: a case report. Br J Ophthalmol 1984;68618- 622
PubMed Link to Article
Pe’er  JHidayat  A Myxomas of the conjunctiva. Am J Ophthalmol 1986;10280- 86
PubMed
Kennedy  RHFlanagan  JCEagle  RC  JrCarney  JA The Carney complex with ocular signs suggestive of cardiac myxoma. Am J Ophthalmol 1991;111699- 702
PubMed
Horie  YIkawa  SNagata  MTamai  A Myxoma of the conjunctiva: a case report and review of the literature. Jpn J Ophthalmol 1995;3977- 82
PubMed
Ramaesh  KWharton  SBDhillon  B Conjunctival myxoma, Zollinger-Ellison syndrome and abnormal thickening of the inter-atrial septum: a case report and review of the literature. Eye 2001;15309- 312
PubMed Link to Article
Maucione  L Di una rara forma di tumore epibulbare (Mixoma puro) suttocongiuntivale. Arch Ottamol 1914;21300- 306
Majewska-Cembrynska  AMajewski  H Sluzak nagalkowy. Klin Oczna 1973;43827- 830
PubMed
El-Rifai  MGhaly  AF Myxoma of the conjunctiva. Bull Ophthalmol Soc Egypt 1975;6831- 35
PubMed
Konari  KSuzuki  JOyachi  H Ocular pain as the initial sign of conjunctival myxoma. Rinsho Ganka 1993;471361- 1364
Burke  APVirmani  R Cardiac myxoma: a clinicopathologic study. Am J Clin Pathol 1993;100671- 680
PubMed
Carney  JA Carney complex: the complex of myxomas, spotty pigmentation, endocrine overactivity, and schwannomas. Semin Dermatol 1995;1490- 98
PubMed Link to Article
Kennedy  RHWaller  RRCarney  JA Ocular pigmented spots and eyelid myxomas. Am J Ophthalmol 1987;104533- 538
PubMed

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