Ophthal Plast Reconstr Surg, Vol. 31, No. 3, 2015
Spindle Cell Lipoma of the Orbit Devjyoti Tripathy, M.S.*, and Ruchi Mittal, M.D., D.N.B.† Abstract: A 30-year-old otherwise healthy female patient presented to the oculoplastic service with complaints of gradually increasing proptosis and discomfort of the OD of about 4 months’ duration. On imaging, an ill-defined mass was seen in the right orbit which, on biopsy, turned out to be a spindle cell lipoma. Lipomas, the commonest among the mesenchymal soft tissue tumors, are surprisingly of rare occurrence in the orbit, despite the presence of abundant fat in the intraorbital space. The authors describe the presentation, management, and outcome of a rare orbital pathology—an orbital spindle cell lipoma.
L
ipomas are the most common soft tissue tumors of adulthood and have several morphological variants.1 Of these, spindle cell lipomas (SCLs) are of rare occurrence and most often arise as subcutaneous, circumscribed masses on the neck, back, and shoulders. SCL of the orbit is distinctly uncommon.2 Herein, the authors report the presentation, management, and outcome in a case of an orbital SCL.
CASE REPORT A 30-year-old female patient presented to the oculoplastic clinic with a history of gradually increasing proptosis and discomfort of the OD of about 4 months’ duration. The best-corrected visual acuity (BCVA) was 20/30, N6 for the OD and 20/20’P’, N6 for the OS. The intraocular pressures were 17 and 15 mm Hg in the OD and OS, respectively. There was no clinical evidence of optic nerve dysfunction on the right side. Posterior segment evaluation showed a mildly hyperemic disc on the right and was unremarkable on the left side. Fullness in the right medial upper eyelid sulcus was noted. Hertel’s exophthalmometry showed a right proptosis of 9 mm, inferotemporal globe dystopia, and ocular motility restriction in adduction and elevation. CT revealed a fairly well-defined heterogeneous fat attenuation mass in the right superomedial orbit extending along the medial orbital wall up to the apex (Figs. 1A and 2). An incisional biopsy of the mass followed by histopathology revealed a predominantly mature adipose tissue component with an interspersed spindle cell element. The spindle cells were arranged in short bundles with bland, plump to elongated nuclei with inconspicuous nucleoli. Interspersed ropey, densely staining short bundles of collagen were noted. Scattered mast cells, mature lymphocytes, and small- to medium-sized vessels were observed. On immunohistochemistry, there was diffuse positivity for CD34. S-100 stained the adipose tissue component, but the spindle cells were negative. Ki-67 showed a positivity of 0.5% to 1.0%. A diagnosis of SCL was thus made (Fig. 3). Considering the risk of optic nerve trauma, a subtotal excision of the tumor was performed with extreme care to ensure only minimal residual at the orbital apex. Postoperative recovery was uneventful. Histopathology showed features almost *Oculoplastics, Orbit and Ocular Oncology Services, and †Ophthalmic Pathology Services, LV Prasad Eye Institute, Bhubaneswar, Odisha, India. Accepted for publication October 30, 2013. Disclosures: The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Devjyoti Tripathy, MS, Oculoplastics, Orbit & Ocular Oncology Services, LV Prasad Eye Institute, Patia, Bhubaneswar 751024, Odisha, India. E-mail [email protected] DOI: 10.1097/IOP.0000000000000068
Case Reports
similar to the incisional biopsy, with more cellular areas and focal nuclear palisading. There was absence of cytologic atypia, and a single mitotic figure was noted after thorough observation by 3 pathologists. Ki-67 was 1% to 2%. Considering consensus opinion, the benign nature of the lesion on histopathology, and excellent vision on the affected side, the patient was not subjected to adjuvant radiation therapy and was kept on close follow up. Eight months postoperatively, the BCVA was 20/20, N6 in the OD with resolution of proptosis (Fig. 1B). There was no optic nerve dysfunction. Ocular motility was restored to normal. One year after excision, the patient continues to remain free of any evidence of recurrence.
DISCUSSION SCLs are benign lipomatous tumors rarely occurring in the orbit. They may be confused with well-differentiated liposarcoma (WDL)/atypical lipomatous tumor (ALT) due to commonalities in clinical and radiological presentation.3 Both are rare tumors in the orbit and can present as painless, slowly progressive proptosis. On CT or MRI, orbital and adnexal SCLs are described to be well-circumscribed masses with variable signal intensities. Liposarcomas, in contrast, are ill-defined, infiltrative masses with heterogeneous tissue densities.3 Heterogeneous tissue density may be a feature of SCL as seen in this case. It is in this context that a biopsy with meticulous histopathological examination assumes paramount importance for accurate diagnosis. WDL/ALT and spindle cell liposarcoma need differentiation from SCL. Floret-like giant cells may be present in SCL; however, the characteristic lipoblast is absent.4,5 Absence of lipoblasts, presence of characteristic ropey collagen, and diffuse staining of CD34 helps to differentiate SCL from liposarcoma.4,5 Murine double minutes (MDM2) immunostaining is of use in differentiating between SCL and WDL/ALT/spindle cell liposarcoma in difficult cases because it is negative in the former.3 Differentiation between SCL and WDL/ALT/spindle cell liposarcoma has important implications regarding clinical management and prognostication. During excision of SCL, a subtotal removal in anatomically critical areas such as the orbital apex (as in our case) may be acceptable due to the extreme rarity of local recurrence. The same would not hold true for liposarcomas where the risk of local recurrence would make it logical to administer postoperative adjuvant radiotherapy. In summary, this report illustrates that SCL of the orbit can be well managed by subtotal surgical excision and a close follow-up protocol. However, it is mandatory to affirm the benign nature of the lesion prior to formulating any treatment strategy and the role of meticulous histopathological evaluation in this cannot be overemphasized.
REFERENCES 1. Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors. 5th ed. St. Louis, MO: Mosby/Elsevier, 2008:429–516. 2. 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. 3. Jakobiec FD, John Nguyen J, Bhat P, et al. MDM2-positive atypical lipomatous neoplasm/well-differentiated diposarcoma versus spindle cell lipoma of the orbit. Ophthal Plast Reconstr Surg 2010;26:413–5. 4. Miettinen MM, Mandahl N. Spindle cell lipoma/pleomorphic lipoma. In: Fletcher CDM, Unni K, Mertens FC, eds.World Health Organization Classification of Tumors, Pathology and Genetics. Tumors of Soft Tissue and Bone. Lyon: IARC Press, 2002:31–2. 5. Dei Tos AP. Liposarcoma: new entities and evolving concepts. Ann Diagn Pathol 2000;4:252–66.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e53
Case Reports
Ophthal Plast Reconstr Surg, Vol. 31, No. 3, 2015
FIG. 1. A, Right superomedial orbital mass (bulge filling out the medial upper eyelid sulcus, asterisk) causing proptosis and inferolateral globe displacement. B, Resolution of proptosis and globe displacement after excision of the mass.
FIG. 2. A, Superior axial, (B) midcoronal, and (C) midsagittal CT scans depicting a fat attenuation mass (asterisks) in the right superomedial orbit extending along the medial wall till the orbital apex.
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© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 31, No. 3, 2015
Case Reports
FIG. 3. A, Low-power photomicrograph (hematoxylin-eosin, ×200) showing irregular distribution of spindle cell areas between mature adipose tissue. B, Photomicrograph showing a hypocellular spindle cell component; the spindle cells are cytologically bland with oval to elongated nuclei and bipolar cytoplasmic processes (hematoxylin-eosin, ×400). C, Diffuse CD34 positivity is evident for the spindle cells (×400). D, S-100 positivity is evident for the adipose tissue with sparing of the spindle cell component (×400).
Psychogenic Unilateral Pseudoptosis Abbas Bagheri, M.D., Ehsan Abbasnia, M.D., Mohammad Pakravan, M.D., Maryam Roshani, M.D., and Mehdi Tavakoli, M.D., F.I.C.O. Abstract: Conversion disorders are among common psychological problems. Pseudoptosis is an uncommon presentation of this category of disorders. Herein, the authors report 2 cases of psychogenic pseudoptosis. The first case is a 21-year-old man who presented with sudden onset of ptosis in his OS 2 weeks previous. The patient reported episodes of left upper eyelid drooping during the last 7 months. The second case is a 10-year-old girl with ptosis onset 6 months previously, after a viral conjunctivitis. In both patients, specific presentations in favor of nonorganic ptosis were observed; all neurological and paraclinical evaluations were normal, and considerable familial or social stressors were found. Both patients received psychological treatment, and no recurrence was observed afterward.
Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Accepted for publication October 20, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Mehdi Tavakoli, M.D.,F.I.C.O., #23, Paydarifard St., Pasdaran Avenue, Tehran 16666, Iran. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000069
C
onversion disorder is defined by DSM-IV as a deficit of sensory or motor function that cannot be explained by a medical condition and where psychological factors are judged to be associated with the deficit because symptoms are preceded by conflicts or other stressors.1 The most common signs of conversion disorder in ophthalmology and neuro-ophthalmology are blindness, diplopia, and gaze palsies. Herein, they report 2 patients with pseudoptosis as a rare manifestation of conversion disorder.2,3 Furthermore, they have discussed about signs and symptoms associated with psychogenic pseudoptosis that help differentiate it from true ptosis.
CASE REPORT The first case was a 21-year-old man who presented with unilateral ptosis. The patient was a worker. He was in low socioeconomic state and married about 8 months ago. There was no history of head or face surgery or trauma. Family history was negative for similar disease. He had sudden-onset ptosis on the left side 2 weeks before presentation. There was no history of fever, trauma, unusual fatigue or weakness of limbs, slurring of speech, voice disorder, or symptoms suggestive of raised intracranial pressure. The history revealed frequent episodes of headache with exacerbation after struggling in family. He remembered periods of ptosis in his left upper eyelid with simultaneous recovery in last 7 months. The amount of ptosis was stable during the daytime. On presentation, examination revealed complete ptosis of the left upper eyelid (Fig. 1A) with no evidence of
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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Spindle Cell Lipoma of the Orbit Devjyoti Tripathy, M.S.*, and Ruchi Mittal, M.D., D.N.B.† Abstract: A 30-year-old otherwise healthy female patient presented to the oculoplastic service with complaints of gradually increasing proptosis and discomfort of the OD of about 4 months’ duration. On imaging, an ill-defined mass was seen in the right orbit which, on biopsy, turned out to be a spindle cell lipoma. Lipomas, the commonest among the mesenchymal soft tissue tumors, are surprisingly of rare occurrence in the orbit, despite the presence of abundant fat in the intraorbital space. The authors describe the presentation, management, and outcome of a rare orbital pathology—an orbital spindle cell lipoma.
L
ipomas are the most common soft tissue tumors of adulthood and have several morphological variants.1 Of these, spindle cell lipomas (SCLs) are of rare occurrence and most often arise as subcutaneous, circumscribed masses on the neck, back, and shoulders. SCL of the orbit is distinctly uncommon.2 Herein, the authors report the presentation, management, and outcome in a case of an orbital SCL.
CASE REPORT A 30-year-old female patient presented to the oculoplastic clinic with a history of gradually increasing proptosis and discomfort of the OD of about 4 months’ duration. The best-corrected visual acuity (BCVA) was 20/30, N6 for the OD and 20/20’P’, N6 for the OS. The intraocular pressures were 17 and 15 mm Hg in the OD and OS, respectively. There was no clinical evidence of optic nerve dysfunction on the right side. Posterior segment evaluation showed a mildly hyperemic disc on the right and was unremarkable on the left side. Fullness in the right medial upper eyelid sulcus was noted. Hertel’s exophthalmometry showed a right proptosis of 9 mm, inferotemporal globe dystopia, and ocular motility restriction in adduction and elevation. CT revealed a fairly well-defined heterogeneous fat attenuation mass in the right superomedial orbit extending along the medial orbital wall up to the apex (Figs. 1A and 2). An incisional biopsy of the mass followed by histopathology revealed a predominantly mature adipose tissue component with an interspersed spindle cell element. The spindle cells were arranged in short bundles with bland, plump to elongated nuclei with inconspicuous nucleoli. Interspersed ropey, densely staining short bundles of collagen were noted. Scattered mast cells, mature lymphocytes, and small- to medium-sized vessels were observed. On immunohistochemistry, there was diffuse positivity for CD34. S-100 stained the adipose tissue component, but the spindle cells were negative. Ki-67 showed a positivity of 0.5% to 1.0%. A diagnosis of SCL was thus made (Fig. 3). Considering the risk of optic nerve trauma, a subtotal excision of the tumor was performed with extreme care to ensure only minimal residual at the orbital apex. Postoperative recovery was uneventful. Histopathology showed features almost *Oculoplastics, Orbit and Ocular Oncology Services, and †Ophthalmic Pathology Services, LV Prasad Eye Institute, Bhubaneswar, Odisha, India. Accepted for publication October 30, 2013. Disclosures: The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Devjyoti Tripathy, MS, Oculoplastics, Orbit & Ocular Oncology Services, LV Prasad Eye Institute, Patia, Bhubaneswar 751024, Odisha, India. E-mail [email protected] DOI: 10.1097/IOP.0000000000000068
Case Reports
similar to the incisional biopsy, with more cellular areas and focal nuclear palisading. There was absence of cytologic atypia, and a single mitotic figure was noted after thorough observation by 3 pathologists. Ki-67 was 1% to 2%. Considering consensus opinion, the benign nature of the lesion on histopathology, and excellent vision on the affected side, the patient was not subjected to adjuvant radiation therapy and was kept on close follow up. Eight months postoperatively, the BCVA was 20/20, N6 in the OD with resolution of proptosis (Fig. 1B). There was no optic nerve dysfunction. Ocular motility was restored to normal. One year after excision, the patient continues to remain free of any evidence of recurrence.
DISCUSSION SCLs are benign lipomatous tumors rarely occurring in the orbit. They may be confused with well-differentiated liposarcoma (WDL)/atypical lipomatous tumor (ALT) due to commonalities in clinical and radiological presentation.3 Both are rare tumors in the orbit and can present as painless, slowly progressive proptosis. On CT or MRI, orbital and adnexal SCLs are described to be well-circumscribed masses with variable signal intensities. Liposarcomas, in contrast, are ill-defined, infiltrative masses with heterogeneous tissue densities.3 Heterogeneous tissue density may be a feature of SCL as seen in this case. It is in this context that a biopsy with meticulous histopathological examination assumes paramount importance for accurate diagnosis. WDL/ALT and spindle cell liposarcoma need differentiation from SCL. Floret-like giant cells may be present in SCL; however, the characteristic lipoblast is absent.4,5 Absence of lipoblasts, presence of characteristic ropey collagen, and diffuse staining of CD34 helps to differentiate SCL from liposarcoma.4,5 Murine double minutes (MDM2) immunostaining is of use in differentiating between SCL and WDL/ALT/spindle cell liposarcoma in difficult cases because it is negative in the former.3 Differentiation between SCL and WDL/ALT/spindle cell liposarcoma has important implications regarding clinical management and prognostication. During excision of SCL, a subtotal removal in anatomically critical areas such as the orbital apex (as in our case) may be acceptable due to the extreme rarity of local recurrence. The same would not hold true for liposarcomas where the risk of local recurrence would make it logical to administer postoperative adjuvant radiotherapy. In summary, this report illustrates that SCL of the orbit can be well managed by subtotal surgical excision and a close follow-up protocol. However, it is mandatory to affirm the benign nature of the lesion prior to formulating any treatment strategy and the role of meticulous histopathological evaluation in this cannot be overemphasized.
REFERENCES 1. Weiss SW, Goldblum JR. Enzinger and Weiss’s Soft Tissue Tumors. 5th ed. St. Louis, MO: Mosby/Elsevier, 2008:429–516. 2. 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. 3. Jakobiec FD, John Nguyen J, Bhat P, et al. MDM2-positive atypical lipomatous neoplasm/well-differentiated diposarcoma versus spindle cell lipoma of the orbit. Ophthal Plast Reconstr Surg 2010;26:413–5. 4. Miettinen MM, Mandahl N. Spindle cell lipoma/pleomorphic lipoma. In: Fletcher CDM, Unni K, Mertens FC, eds.World Health Organization Classification of Tumors, Pathology and Genetics. Tumors of Soft Tissue and Bone. Lyon: IARC Press, 2002:31–2. 5. Dei Tos AP. Liposarcoma: new entities and evolving concepts. Ann Diagn Pathol 2000;4:252–66.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e53
Case Reports
Ophthal Plast Reconstr Surg, Vol. 31, No. 3, 2015
FIG. 1. A, Right superomedial orbital mass (bulge filling out the medial upper eyelid sulcus, asterisk) causing proptosis and inferolateral globe displacement. B, Resolution of proptosis and globe displacement after excision of the mass.
FIG. 2. A, Superior axial, (B) midcoronal, and (C) midsagittal CT scans depicting a fat attenuation mass (asterisks) in the right superomedial orbit extending along the medial wall till the orbital apex.
e54
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 31, No. 3, 2015
Case Reports
FIG. 3. A, Low-power photomicrograph (hematoxylin-eosin, ×200) showing irregular distribution of spindle cell areas between mature adipose tissue. B, Photomicrograph showing a hypocellular spindle cell component; the spindle cells are cytologically bland with oval to elongated nuclei and bipolar cytoplasmic processes (hematoxylin-eosin, ×400). C, Diffuse CD34 positivity is evident for the spindle cells (×400). D, S-100 positivity is evident for the adipose tissue with sparing of the spindle cell component (×400).
Psychogenic Unilateral Pseudoptosis Abbas Bagheri, M.D., Ehsan Abbasnia, M.D., Mohammad Pakravan, M.D., Maryam Roshani, M.D., and Mehdi Tavakoli, M.D., F.I.C.O. Abstract: Conversion disorders are among common psychological problems. Pseudoptosis is an uncommon presentation of this category of disorders. Herein, the authors report 2 cases of psychogenic pseudoptosis. The first case is a 21-year-old man who presented with sudden onset of ptosis in his OS 2 weeks previous. The patient reported episodes of left upper eyelid drooping during the last 7 months. The second case is a 10-year-old girl with ptosis onset 6 months previously, after a viral conjunctivitis. In both patients, specific presentations in favor of nonorganic ptosis were observed; all neurological and paraclinical evaluations were normal, and considerable familial or social stressors were found. Both patients received psychological treatment, and no recurrence was observed afterward.
Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran. Accepted for publication October 20, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Mehdi Tavakoli, M.D.,F.I.C.O., #23, Paydarifard St., Pasdaran Avenue, Tehran 16666, Iran. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000069
C
onversion disorder is defined by DSM-IV as a deficit of sensory or motor function that cannot be explained by a medical condition and where psychological factors are judged to be associated with the deficit because symptoms are preceded by conflicts or other stressors.1 The most common signs of conversion disorder in ophthalmology and neuro-ophthalmology are blindness, diplopia, and gaze palsies. Herein, they report 2 patients with pseudoptosis as a rare manifestation of conversion disorder.2,3 Furthermore, they have discussed about signs and symptoms associated with psychogenic pseudoptosis that help differentiate it from true ptosis.
CASE REPORT The first case was a 21-year-old man who presented with unilateral ptosis. The patient was a worker. He was in low socioeconomic state and married about 8 months ago. There was no history of head or face surgery or trauma. Family history was negative for similar disease. He had sudden-onset ptosis on the left side 2 weeks before presentation. There was no history of fever, trauma, unusual fatigue or weakness of limbs, slurring of speech, voice disorder, or symptoms suggestive of raised intracranial pressure. The history revealed frequent episodes of headache with exacerbation after struggling in family. He remembered periods of ptosis in his left upper eyelid with simultaneous recovery in last 7 months. The amount of ptosis was stable during the daytime. On presentation, examination revealed complete ptosis of the left upper eyelid (Fig. 1A) with no evidence of
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e55
