Case Reports “Low Fat” Spindle Cell Lipoma of the Eyelid: A Diagnostic Challenge Norman C. Charles, M.D., and Irina Belinsky, M.D. Abstract: The authors describe a spindle cell lipoma that occurred in the anterior lamellae of the eyelid in a 47-yearold man. This benign tumor was reported previously only once within that location and poses histologic challenges when a “low fat” or “fat free” variant is present.
S
pindle cell lipomas are rare tumors of the ocular adnexa that involve the orbit and eyelid. The authors describe an unusual variant of this tumor within the eyelid that posed a challenge for histologic interpretation. This case report is compliant with HIPAA guidelines.
FIG. 1. A, Computed tomography, sagittal view shows low density mass (asterisk). B, Intraoperative bulge is outlined by dye.
CASE REPORT A healthy 47-year-old man noted an enlarging painless mass of the right lower eyelid over 1 year. He denied experiencing diplopia, previous injury or surgery. There was no personal or familial history of cancer. Uncorrected vision was 20/20 OU. There was a nontender, mobile 2.5 × 1.5 cm mass of the right lower eyelid that approximated the eyelid margin. No other ocular abnormalities were present. Computerized tomography delineated a low density lesion in the right lower eyelid extending to the premaxillary region (Fig. 1). Surgical transconjunctival exploration via the inferior fornix revealed a discrete, well-demarcated tumor in the preseptal plane. Gross examination showed a brown, rubbery, ovoid mass measuring approximately 2.2 × 1.4 × 1.2 cm. Histologic sections disclosed a myxoid intramuscular tumor that contained round-to-spindle shaped, short, stubby nuclei with indistinct, granular mucinous cytoplasm (Fig. 2A). A diffuse background network of ropey, refractile collagen bundles was present. One portion of the tumor was relatively hypercellular. Scattered mast cells and only rare adipocytes were noted (Fig. 2B). There was diffuse tumor cell positivity for CD34, vimentin, and bcl2 immunostains. Scattered positivity for progesterone receptors and CD99 was present. Negative markers included Rb, S-100, cytokeratins AE1/AE and CAM5.2, smooth muscle actin, desmin, STAT6, and MDM2. Ki67 showed a low proliferation rate.
DISCUSSION Initial histologic diagnoses included myxoma, myxofibroma, myxoid variant of solitary fibrous tumor, and bland fibromyxoid neoplasm. On further consultation, Dr. Christopher DM Fletcher noted that the diffuse positivity of the lesional spindle cells for CD34 and the negativity for Rb supported the diagnosis of low fat spindle cell lipoma.1 First described by Enzinger and Harvey,2 spindle cell lipomas are benign, painless, slowly growing subcutaneous tumors Accepted for publication March 26, 2015. Departments of Ophthalmology and Pathology, New York University Langone Medical Center, New York, New York, U.S.A. Presented at the Eastern Ophthalmic Pathology Society Meeting on November 6–8, 2014 in Baltimore, MD. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Norman C. Charles, M.D., New York University Langone Medical Center, 550 First Avenue, New York, NY 10016. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000492
Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2015
FIG. 2. A, Tumor consists of round-to-spindle shaped nuclei amidst myxoid background (hematoxylin-eosin, original magnification ×12, left). Higher power shows ropey, pink collagenous fibrillar network (arrowheads) amidst tumor cells and mucinous background (m) (hematoxylin-eosin, original magnification ×300, right). B, CD34 immunoperoxidase reaction, diaminobenzine chromogen, original magnification ×200. Inset Rare adipocytes with clear cytoplasm (asterisks) interposed between tumor and skeletal muscle fibers (hematoxylin-eosin, original magnification ×400).
that occur in the back, shoulders, and posterior neck with a predilection for middle-aged men.3 Superficial facial lesions, such as the current one, may extend into skeletal muscle. Rare cases have occurred in the orbit4–7 and only 1 previous case has been reported in the eyelid.8 Occasional multiple, familial lesions have been documented. Spindle cell lipomas and pleomorphic
e1
Copyright © 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2015
Case Reports
lipomas share clinical and histological features they are considered as parts of a pathologic spectrum.9 These well-circumscribed, thinly encapsulated lesions contain variable proportions of mature adipocytes and undifferentiated CD34-positive, S-100 negative spindle cells within a background of myxoid stroma and scattered mast cells. Diagnosis can be elusive when “low fat” or “fat-free” histologic patterns occur.10 Unlike the CD34-positive myxoid variety of solitary fibrous tumor, these tumor cells lack the Rb gene product and the solitary fibrous tumor-specific STAT6 expression.1,11 Palisading of tumor cells may simulate schwannoma, but S-100 negativity militates against that interpretation. Spindle cell lipoma’s low Ki67 proliferation rate and MDM2 negativity distinguishes it from CD34-positive atypical lipomatous neoplasm/ well-differentiated liposarcoma as the latter tumor is MDM2 positive with a high proliferation rate.12 Lesions consisting of pure adipocytic content are termed lipomas and have rarely been described in the eyelid.13,14 The sole previous case of eyelid spindle cell lipoma contained abundant fat cells admixed with spindle cells, facilitating the diagnosis.8 The current “fat-free configuration” presenting within the eyelid is unique.
ACKNOWLEDGMENTS We are grateful to Christopher D.M. Fletcher, MD who kindly reviewed the histology and established the diagnosis. Payal Patel, MD supervised the surgery.
REFERENCES 1. Chen BJ, Mariño-Enríquez A, Fletcher CD, et al. Loss of retinoblastoma protein expression in spindle cell/pleomorphic lipomas
e2
and cytogenetically related tumors: an immunohistochemical study with diagnostic implications. Am J Surg Pathol 2012;36:1119–28. 2. Enzinger FM, Harvey DA. Spindle cell lipoma. Cancer 1975;36:1852–9. 3. Goldblum JR, Folpe Al, Weiss SW. Chap 14 Benign Lipomatous Tumors in Enzinger and Weiss’s Soft Tissue Tumors. 6th ed. Philadelphia, PA: Elsevier/Saunders, 2014:456–63. 4. Johnson BL, Linn JG, Jr. Spindle cell lipoma of the orbit. Arch Ophthalmol 1979;97:133–4. 5. Bartley GB, Yeatts RP, Garrity JA, et al. Spindle cell lipoma of the orbit. Am J Ophthalmol 1985;100:605–9. 6. Ulivieri S, Olivieri G, Motolese PA, et al. Spindle cell lipoma of the orbit: a case report of an unusual orbital pathology. Neurol Neurochir Pol 2010;44:419–23. 7. Tripathi D, Mittal R. Spindle cell lipoma of the orbit. Ophthal Plast Reconstr Surg 2014;20:e1–e3. 8. Mawn LA, Jordan DR, Olberg B. Spindle-cell lipoma of the preseptal eyelid. Ophthal Plast Reconstr Surg 1998;14:174–7. 9. Fletcher CDM. Diagnostic Histopathology of Tumors. Vol 2. 2nd ed. London, England: Churchill Livingstone, 2000. 10. Billings SD, Folpe AL. Diagnostically challenging spindle cell lipomas: a report of 34 “low-fat” and “fat-free” variants. Am J Dermatopathol 2007;29:437–42. 11. Doyle LA, Vivero M, Fletcher CD, et al. Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics. Mod Pathol 2014;27:390–5. 12. Jakobiec FA, Nguyen J, Bhat P, et al. MDM2-positive atypi cal lipomatous neoplasm/well-differentiated liposarcoma versus spindle cell lipoma of the orbit. Ophthal Plast Reconstr Surg 2010;26:413–5. 13. Charles NC, Palu RN. Intramuscular lipoma of the eyelid. Ophthalmic Surg Lasers 2000;31:340–1. 14. Thyparampil P, Diwan AH, Diaz-Marchan P, et al. Eyelid lipomas: a case report and review of the literature. Orbit 2012;31:319–20.
© 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Copyright © 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
S
pindle cell lipomas are rare tumors of the ocular adnexa that involve the orbit and eyelid. The authors describe an unusual variant of this tumor within the eyelid that posed a challenge for histologic interpretation. This case report is compliant with HIPAA guidelines.
FIG. 1. A, Computed tomography, sagittal view shows low density mass (asterisk). B, Intraoperative bulge is outlined by dye.
CASE REPORT A healthy 47-year-old man noted an enlarging painless mass of the right lower eyelid over 1 year. He denied experiencing diplopia, previous injury or surgery. There was no personal or familial history of cancer. Uncorrected vision was 20/20 OU. There was a nontender, mobile 2.5 × 1.5 cm mass of the right lower eyelid that approximated the eyelid margin. No other ocular abnormalities were present. Computerized tomography delineated a low density lesion in the right lower eyelid extending to the premaxillary region (Fig. 1). Surgical transconjunctival exploration via the inferior fornix revealed a discrete, well-demarcated tumor in the preseptal plane. Gross examination showed a brown, rubbery, ovoid mass measuring approximately 2.2 × 1.4 × 1.2 cm. Histologic sections disclosed a myxoid intramuscular tumor that contained round-to-spindle shaped, short, stubby nuclei with indistinct, granular mucinous cytoplasm (Fig. 2A). A diffuse background network of ropey, refractile collagen bundles was present. One portion of the tumor was relatively hypercellular. Scattered mast cells and only rare adipocytes were noted (Fig. 2B). There was diffuse tumor cell positivity for CD34, vimentin, and bcl2 immunostains. Scattered positivity for progesterone receptors and CD99 was present. Negative markers included Rb, S-100, cytokeratins AE1/AE and CAM5.2, smooth muscle actin, desmin, STAT6, and MDM2. Ki67 showed a low proliferation rate.
DISCUSSION Initial histologic diagnoses included myxoma, myxofibroma, myxoid variant of solitary fibrous tumor, and bland fibromyxoid neoplasm. On further consultation, Dr. Christopher DM Fletcher noted that the diffuse positivity of the lesional spindle cells for CD34 and the negativity for Rb supported the diagnosis of low fat spindle cell lipoma.1 First described by Enzinger and Harvey,2 spindle cell lipomas are benign, painless, slowly growing subcutaneous tumors Accepted for publication March 26, 2015. Departments of Ophthalmology and Pathology, New York University Langone Medical Center, New York, New York, U.S.A. Presented at the Eastern Ophthalmic Pathology Society Meeting on November 6–8, 2014 in Baltimore, MD. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Norman C. Charles, M.D., New York University Langone Medical Center, 550 First Avenue, New York, NY 10016. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000492
Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2015
FIG. 2. A, Tumor consists of round-to-spindle shaped nuclei amidst myxoid background (hematoxylin-eosin, original magnification ×12, left). Higher power shows ropey, pink collagenous fibrillar network (arrowheads) amidst tumor cells and mucinous background (m) (hematoxylin-eosin, original magnification ×300, right). B, CD34 immunoperoxidase reaction, diaminobenzine chromogen, original magnification ×200. Inset Rare adipocytes with clear cytoplasm (asterisks) interposed between tumor and skeletal muscle fibers (hematoxylin-eosin, original magnification ×400).
that occur in the back, shoulders, and posterior neck with a predilection for middle-aged men.3 Superficial facial lesions, such as the current one, may extend into skeletal muscle. Rare cases have occurred in the orbit4–7 and only 1 previous case has been reported in the eyelid.8 Occasional multiple, familial lesions have been documented. Spindle cell lipomas and pleomorphic
e1
Copyright © 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
Ophthal Plast Reconstr Surg, Vol. XX, No. XX, 2015
Case Reports
lipomas share clinical and histological features they are considered as parts of a pathologic spectrum.9 These well-circumscribed, thinly encapsulated lesions contain variable proportions of mature adipocytes and undifferentiated CD34-positive, S-100 negative spindle cells within a background of myxoid stroma and scattered mast cells. Diagnosis can be elusive when “low fat” or “fat-free” histologic patterns occur.10 Unlike the CD34-positive myxoid variety of solitary fibrous tumor, these tumor cells lack the Rb gene product and the solitary fibrous tumor-specific STAT6 expression.1,11 Palisading of tumor cells may simulate schwannoma, but S-100 negativity militates against that interpretation. Spindle cell lipoma’s low Ki67 proliferation rate and MDM2 negativity distinguishes it from CD34-positive atypical lipomatous neoplasm/ well-differentiated liposarcoma as the latter tumor is MDM2 positive with a high proliferation rate.12 Lesions consisting of pure adipocytic content are termed lipomas and have rarely been described in the eyelid.13,14 The sole previous case of eyelid spindle cell lipoma contained abundant fat cells admixed with spindle cells, facilitating the diagnosis.8 The current “fat-free configuration” presenting within the eyelid is unique.
ACKNOWLEDGMENTS We are grateful to Christopher D.M. Fletcher, MD who kindly reviewed the histology and established the diagnosis. Payal Patel, MD supervised the surgery.
REFERENCES 1. Chen BJ, Mariño-Enríquez A, Fletcher CD, et al. Loss of retinoblastoma protein expression in spindle cell/pleomorphic lipomas
e2
and cytogenetically related tumors: an immunohistochemical study with diagnostic implications. Am J Surg Pathol 2012;36:1119–28. 2. Enzinger FM, Harvey DA. Spindle cell lipoma. Cancer 1975;36:1852–9. 3. Goldblum JR, Folpe Al, Weiss SW. Chap 14 Benign Lipomatous Tumors in Enzinger and Weiss’s Soft Tissue Tumors. 6th ed. Philadelphia, PA: Elsevier/Saunders, 2014:456–63. 4. Johnson BL, Linn JG, Jr. Spindle cell lipoma of the orbit. Arch Ophthalmol 1979;97:133–4. 5. Bartley GB, Yeatts RP, Garrity JA, et al. Spindle cell lipoma of the orbit. Am J Ophthalmol 1985;100:605–9. 6. Ulivieri S, Olivieri G, Motolese PA, et al. Spindle cell lipoma of the orbit: a case report of an unusual orbital pathology. Neurol Neurochir Pol 2010;44:419–23. 7. Tripathi D, Mittal R. Spindle cell lipoma of the orbit. Ophthal Plast Reconstr Surg 2014;20:e1–e3. 8. Mawn LA, Jordan DR, Olberg B. Spindle-cell lipoma of the preseptal eyelid. Ophthal Plast Reconstr Surg 1998;14:174–7. 9. Fletcher CDM. Diagnostic Histopathology of Tumors. Vol 2. 2nd ed. London, England: Churchill Livingstone, 2000. 10. Billings SD, Folpe AL. Diagnostically challenging spindle cell lipomas: a report of 34 “low-fat” and “fat-free” variants. Am J Dermatopathol 2007;29:437–42. 11. Doyle LA, Vivero M, Fletcher CD, et al. Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics. Mod Pathol 2014;27:390–5. 12. Jakobiec FA, Nguyen J, Bhat P, et al. MDM2-positive atypi cal lipomatous neoplasm/well-differentiated liposarcoma versus spindle cell lipoma of the orbit. Ophthal Plast Reconstr Surg 2010;26:413–5. 13. Charles NC, Palu RN. Intramuscular lipoma of the eyelid. Ophthalmic Surg Lasers 2000;31:340–1. 14. Thyparampil P, Diwan AH, Diaz-Marchan P, et al. Eyelid lipomas: a case report and review of the literature. Orbit 2012;31:319–20.
© 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Copyright © 2015 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Unauthorized reproduction of this article is prohibited.
