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Orbital apex leiomyoma with intracranial extension
Orbital Apex Leiomyoma with Intracranial Extension Vaijayantee Kulkarni, M.Ch.,* Vedantam Rajshekhar, M.Ch.,* and Sushil M. Chandi, M.D.† *Departments of Neurological Sciences and †Pathology, Christian Medical College and Hospital, Vellore, India
Kulkarni V, Rajshekhar V, Chandi S. Orbital apex leiomyoma with intracranial extension. Surg Neurol 2000;54:327–30. BACKGROUND
Leiomyoma is a rare, benign smooth muscle tumor of the orbit. It occasionally shows some histologic resemblance to other common tumors of the orbit like neurofibroma and schwannoma. Its location at the orbital apex is uncommon and only one case with intracranial extension has been reported so far. CASE DESCRIPTION
A nine-year-old boy presented to us with left orbital pain. Four years earlier he had undergone partial excision, elsewhere, of a tumor at the orbital apex, which was reported as a schwannoma. The computed tomography (CT) scan showed regrowth of the tumor with intracranial extension. The tumor was totally resected by an intracranial route. One year postoperatively there was no recurrence of the tumor. CONCLUSIONS
Leiomyoma of the orbit, though a benign tumor, does show regrowth after partial excision. Total excision, including any intracranial component, is advised. The cases reported so far are reviewed and the histopathology and possible etiopathogenesis of this tumor are discussed. © 2000 by Elsevier Science Inc. KEY WORDS
Orbital tumor, leiomyoma.
eiomyoma is a rare tumor of the orbit and very few cases have been reported so far [1,3,4,6,7]. Its location at the orbital apex is rarer still and intracranial extension has been previously documented in only one case [6]. We report a case of leiomyoma of the orbital apex with intracranial extension through the superior orbital fissure, which was initially diagnosed as a schwannoma and showed regrowth after partial excision.
L
Address reprint requests to: Dr V. Rajshekhar, Department of Neurological Sciences, Christian Medical College and Hospital, Vellore 632004, India. Received July 12, 2000; accepted August 9, 2000. © 2000 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010
Case Report A nine-year-old boy presented with increasing left retro-orbital headache of 8 months’ duration. He had not noticed diplopia or diminution of vision. There was no history of facial numbness, seizures, or symptoms of raised intracranial pressure. There was no family history suggestive of neurofibromatosis. Four years earlier he had developed progressive left lateral rectus paresis and mild proptosis. The computed tomography (CT) scan had revealed a left orbital apex tumor with intracranial extension. Elsewhere, he had undergone partial excision of the tumor by a transcranial route. The tumor was reported as a schwannoma. Postoperatively he developed sixth nerve palsy that gradually improved. A postoperative CT scan showed residual tumor at the orbital apex with an intracranial component. He had mild headache that persisted and increased at the time of presentation. The proptosis had remained unchanged. On examination, there were no signs of neurofibromatosis. There was a well healed scar from a left frontal craniotomy. There was mild axial proptosis of the left eye. Eye movements were full. Visual acuity and visual fields were bilaterally normal. The left optic disc showed dilated veins and absent venous pulsations while the right optic disc was normal. There were no neurological deficits. An X-ray of the skull (anteroposterior view) showed elevation of the lesser sphenoid wing and widening of the left superior orbital fissure. CT scan showed a well-defined mass at the left orbital apex and in the region of the left superior orbital fissure (Figure 1). There was a significant increase in the size of the residual tumor as compared to the postoperative scan taken 4 years earlier. From the orbital apex there was anterior intra-conal extension of the tumor in the posterior part of the orbit. The optic nerve was buckled and pushed medially and 0090-3019/00/$–see front matter PII S0090-3019(00)00310-4
328 Surg Neurol 2000;54:327–30
Kulkarni et al
A contrast enhanced CT scan of the brain, axial (A) and coronal (B) views, showing an enhancing mass at the left orbital apex pushing the optic nerve medially. Note the extension of the tumor into the middle cranial fossa through the superior orbital fissure.
1
there was remolding and elevation of the lesser wing of the sphenoid resulting in widening of the superior orbital fissure. Posteriorly the tumor extended into the middle cranial fossa at the temporal pole. The suprasellar cistern was normal. A left fronto-temporal craniotomy was performed and the tumor was totally excised. The roof of the orbit appeared thinned out and was eroded near the apex. There was tumor filling the left orbital apex and extending posteriorly through the superior orbital fissure into the extradural space in the middle cranial fossa. The sphenoid ridge and the greater wing of the sphenoid forming the walls of the superior orbital fissure were drilled to gain access to the tumor. The tumor was avascular, firm to hard in consistency, and appeared encapsulated. It was separated from the orbital contents including the optic nerve by the orbital fat and from the cranial nerves in the superior orbital fissure by a fascial plane. Histopathological examination showed the tumor to be composed of interwoven fascicles of benign smooth muscle cells with a whorled pattern, enveloped in hyalinised fibrocollagenous tissue, and was diagnostic of leiomyoma (Figure 2). Postoperatively the patient developed transient
partial third nerve paresis, which recovered rapidly. A postoperative contrast enhanced CT scan showed no residual tumor (Figure 3). Patient was asymptomatic at follow-up a year later, and the CT scan showed no recurrence of the tumor (Figure 4).
Discussion Leiomyoma, a benign tumor arising from the smooth muscle cells, is an uncommon tumor of the orbit. Since its initial description by Lodato in 1896, to our knowledge, not more than 20 cases have been reported [1]. The majority of these patients have been young adults in the first two decades with no sex predisposition. Most of the tumors have been located in the anterior part of the orbit, with slight a predilection for the inferior and medial quadrants [1,3,4,7]. Clinically they cause painless proptosis or displacement of the globe, progressing slowly over several months or years. HISTOPATHOLOGY The classical histological features of leiomyoma include bundles of spindle-shaped cells arranged in whorls; elongated, blunt-tipped, cigar-shaped nu-
Orbital Leiomyoma
Surg Neurol 329 2000;54:327–30
A contrast-enhanced CT scan of the brain conducted 1 year postoperatively shows no tumor recurrence.
4
trichrome staining may be useful in arriving at the diagnosis. Electron microscopy (EM) is recommended in doubtful cases [5,7]. Photomicrograph showing sheets of spindle shaped cells arranged in a whorled pattern, typical of a leiomyoma. (H & E ⫻ 200).
2
clei; retraction of cytoplasm around the nucleus; and cytoplasmic eosinophilia [3,4]. Neurofibroma, fibrous histiocytoma, schwannoma, and amelanotic melanoma have a similar histologic appearance on routine haematoxylin-eosin stain, but Masson’s
Postoperative contrast CT scan of the brain showing total excision of the orbital mass and its intracranial extension.
3
HISTOPATHOGENESIS The origin of these smooth muscle tumors of the orbit has been a matter of speculation. Nath et al [6] suggested the capsulo-palpebral muscle of Hessar as the probable source of its histogenesis. Orbital leiomyomas constitute a spectrum of tumors ranging from vascular leiomyoma or angiomyoma with a large component of capillaries to a rather solid smooth muscle tumor that does not feature a conspicuous vascular component [2]. Because of the occurrence of vascular leiomyomas, the vascular smooth muscles in the vessel walls and the pericytes are also proposed as the source of origin of this tumor in the orbit [3]. In our case, the tumor extended intracranially through the superior orbital fissure; therefore, a vascular origin seems likely, because many of the ophthalmic veins pass through the superior orbital fissure. INTRACRANIAL EXTENSION AND RECURRENCE Jakobiec et al [4] reported a 34-year-old woman with an orbital leiomyoma that was partially excised through a lateral canthotomy, leaving behind the part that was adherent at the superior orbital fissure. Though widening of the superior orbital fissure was seen on X-ray, the extent of intracranial extension was not defined. The residue subsequently showed regrowth 7 years later and developed sarcomatous change 6 years after radiother-
330 Surg Neurol 2000;54:327–30
1
Kulkarni et al
Features of Some Reported Cases of Orbital Leiomyoma
LOCATION
INTRACRANIAL
AGE/SEX
AUTHOR/YEAR
IN ORBIT
EXTENSION
OF RESECTION
18/F 17/M 17/M 34/M
Nath & Shukla 1962 Jacobiec et al 1975 Jacobiec et al 1975 Jacobiec et al 1975
Apex Center Ant/med Apex
No No No Present
Total Partial Total Partial
5/M 39/M
Sanborn et al 1979 Betharia et al 1991
Sup. Ant.
No No
Total Total
apy. Nath et al [6] reported one case of orbital apex leiomyoma that was excised via a trans-orbital route. There was no intracranial extension. The natural history of these tumors in the orbit is not known. The location of the tumor in the orbit, extent of resection, and recurrence in some of the reported cases are shown in Table 1. No recurrence has been reported after total excision of an orbital leiomyoma; however, regrowth after partial excision has been reported in two cases [3,4]. In our case too, the tumor showed a significant increase in size, especially of the intracranial component, over a 4-year period after partial resection. Unlike previously reported cases, the tumor was adequately imaged, and was approached by a transcranial and lateral orbital route and was totally excised. The intracranial tumor was totally extradural, within the superior orbital fissure, but did not extend into the cavernous sinus. The tumor was found to be separated from nerves by a fascial plane, which enabled total excision without significant morbidity.
Conclusion Leiomyoma of the posterior orbit with intracranial extension is a very rare entity, and should be differentiated from other common tumors of the orbit like schwannoma and neurofibroma on histology. It can regrow after partial excision, is radioresistant, and may show sarcomatous change. Therefore, total excision by a transcranial route is advised. REFERENCES 1. Betharia SM, Arora R, Kishore K, Patil ND. Leiomyoma of the orbit. Ind J Ophthal 1991;39:35–7.
EXTENT
RECURRENCE No Progression after 6 yrs No Regrowth after 7 years, sarcomatous change after radiotherapy No No
2. Cherrik HM, Dunlap C, King O. Leiomyoma of the oral cavity. Review of literature and clinicopathologic study of seven new cases. Oral Surg 1973;35:54 –57. 3. Jacobiec FA, Jones IS, Tannenbaum M. Leiomyoma: an unusual tumor of the orbit. Br J Ophthalmol 1973;57: 825–31. 4. Jacobiec FA, Howard GM, Rosen M, Wolff M. Leiomyoma and leiomyosarcoma of the orbit. Am J Ophthalmol 1975;80:1028 – 42. 5. Meyer SL, Fine BS, Font RL, Zimmerman LE. Leiomyoma of the ciliary body: electron microscopic verification. Am J Ophthalmol 1968;66:1061– 68. 6. Nath K, Shukla BR. Orbital leiomyoma and its origin. Br J Ophthalmol 1963;47:369 –71. 7. Sanborn GE, Valenzuela RE, Green WR. Leiomyoma of the orbit. Am J Ophthalmol 1979;87:371–75.
COMMENTARY
Leiomyoma of the orbit is indeed a rare tumor. In our series of over 1,500 orbital tumors, we have not diagnosed or operated on one of these. It would certainly be easy to mistake this on neuroimaging for a neurofibroma or even a meningioma, but as the authors point out, the tumor was completely encapsulated and was removed in a fairly straightforward fashion, with preservation of vision. The authors emphasize that total excision should be done, if at all possible, at the first procedure, since—as demonstrated by this case—they do recur. This rare but curable tumor should be kept in mind when considering the differential diagnosis of orbital apex neoplasms. Joseph C. Maroon, M.D. Department of Neurosurgery University of Pittsburgh Medical Center Pittsburgh, Pennsylvania
Kulkarni V, Rajshekhar V, Chandi S. Orbital apex leiomyoma with intracranial extension. Surg Neurol 2000;54:327–30. BACKGROUND
Leiomyoma is a rare, benign smooth muscle tumor of the orbit. It occasionally shows some histologic resemblance to other common tumors of the orbit like neurofibroma and schwannoma. Its location at the orbital apex is uncommon and only one case with intracranial extension has been reported so far. CASE DESCRIPTION
A nine-year-old boy presented to us with left orbital pain. Four years earlier he had undergone partial excision, elsewhere, of a tumor at the orbital apex, which was reported as a schwannoma. The computed tomography (CT) scan showed regrowth of the tumor with intracranial extension. The tumor was totally resected by an intracranial route. One year postoperatively there was no recurrence of the tumor. CONCLUSIONS
Leiomyoma of the orbit, though a benign tumor, does show regrowth after partial excision. Total excision, including any intracranial component, is advised. The cases reported so far are reviewed and the histopathology and possible etiopathogenesis of this tumor are discussed. © 2000 by Elsevier Science Inc. KEY WORDS
Orbital tumor, leiomyoma.
eiomyoma is a rare tumor of the orbit and very few cases have been reported so far [1,3,4,6,7]. Its location at the orbital apex is rarer still and intracranial extension has been previously documented in only one case [6]. We report a case of leiomyoma of the orbital apex with intracranial extension through the superior orbital fissure, which was initially diagnosed as a schwannoma and showed regrowth after partial excision.
L
Address reprint requests to: Dr V. Rajshekhar, Department of Neurological Sciences, Christian Medical College and Hospital, Vellore 632004, India. Received July 12, 2000; accepted August 9, 2000. © 2000 by Elsevier Science Inc. 655 Avenue of the Americas, New York, NY 10010
Case Report A nine-year-old boy presented with increasing left retro-orbital headache of 8 months’ duration. He had not noticed diplopia or diminution of vision. There was no history of facial numbness, seizures, or symptoms of raised intracranial pressure. There was no family history suggestive of neurofibromatosis. Four years earlier he had developed progressive left lateral rectus paresis and mild proptosis. The computed tomography (CT) scan had revealed a left orbital apex tumor with intracranial extension. Elsewhere, he had undergone partial excision of the tumor by a transcranial route. The tumor was reported as a schwannoma. Postoperatively he developed sixth nerve palsy that gradually improved. A postoperative CT scan showed residual tumor at the orbital apex with an intracranial component. He had mild headache that persisted and increased at the time of presentation. The proptosis had remained unchanged. On examination, there were no signs of neurofibromatosis. There was a well healed scar from a left frontal craniotomy. There was mild axial proptosis of the left eye. Eye movements were full. Visual acuity and visual fields were bilaterally normal. The left optic disc showed dilated veins and absent venous pulsations while the right optic disc was normal. There were no neurological deficits. An X-ray of the skull (anteroposterior view) showed elevation of the lesser sphenoid wing and widening of the left superior orbital fissure. CT scan showed a well-defined mass at the left orbital apex and in the region of the left superior orbital fissure (Figure 1). There was a significant increase in the size of the residual tumor as compared to the postoperative scan taken 4 years earlier. From the orbital apex there was anterior intra-conal extension of the tumor in the posterior part of the orbit. The optic nerve was buckled and pushed medially and 0090-3019/00/$–see front matter PII S0090-3019(00)00310-4
328 Surg Neurol 2000;54:327–30
Kulkarni et al
A contrast enhanced CT scan of the brain, axial (A) and coronal (B) views, showing an enhancing mass at the left orbital apex pushing the optic nerve medially. Note the extension of the tumor into the middle cranial fossa through the superior orbital fissure.
1
there was remolding and elevation of the lesser wing of the sphenoid resulting in widening of the superior orbital fissure. Posteriorly the tumor extended into the middle cranial fossa at the temporal pole. The suprasellar cistern was normal. A left fronto-temporal craniotomy was performed and the tumor was totally excised. The roof of the orbit appeared thinned out and was eroded near the apex. There was tumor filling the left orbital apex and extending posteriorly through the superior orbital fissure into the extradural space in the middle cranial fossa. The sphenoid ridge and the greater wing of the sphenoid forming the walls of the superior orbital fissure were drilled to gain access to the tumor. The tumor was avascular, firm to hard in consistency, and appeared encapsulated. It was separated from the orbital contents including the optic nerve by the orbital fat and from the cranial nerves in the superior orbital fissure by a fascial plane. Histopathological examination showed the tumor to be composed of interwoven fascicles of benign smooth muscle cells with a whorled pattern, enveloped in hyalinised fibrocollagenous tissue, and was diagnostic of leiomyoma (Figure 2). Postoperatively the patient developed transient
partial third nerve paresis, which recovered rapidly. A postoperative contrast enhanced CT scan showed no residual tumor (Figure 3). Patient was asymptomatic at follow-up a year later, and the CT scan showed no recurrence of the tumor (Figure 4).
Discussion Leiomyoma, a benign tumor arising from the smooth muscle cells, is an uncommon tumor of the orbit. Since its initial description by Lodato in 1896, to our knowledge, not more than 20 cases have been reported [1]. The majority of these patients have been young adults in the first two decades with no sex predisposition. Most of the tumors have been located in the anterior part of the orbit, with slight a predilection for the inferior and medial quadrants [1,3,4,7]. Clinically they cause painless proptosis or displacement of the globe, progressing slowly over several months or years. HISTOPATHOLOGY The classical histological features of leiomyoma include bundles of spindle-shaped cells arranged in whorls; elongated, blunt-tipped, cigar-shaped nu-
Orbital Leiomyoma
Surg Neurol 329 2000;54:327–30
A contrast-enhanced CT scan of the brain conducted 1 year postoperatively shows no tumor recurrence.
4
trichrome staining may be useful in arriving at the diagnosis. Electron microscopy (EM) is recommended in doubtful cases [5,7]. Photomicrograph showing sheets of spindle shaped cells arranged in a whorled pattern, typical of a leiomyoma. (H & E ⫻ 200).
2
clei; retraction of cytoplasm around the nucleus; and cytoplasmic eosinophilia [3,4]. Neurofibroma, fibrous histiocytoma, schwannoma, and amelanotic melanoma have a similar histologic appearance on routine haematoxylin-eosin stain, but Masson’s
Postoperative contrast CT scan of the brain showing total excision of the orbital mass and its intracranial extension.
3
HISTOPATHOGENESIS The origin of these smooth muscle tumors of the orbit has been a matter of speculation. Nath et al [6] suggested the capsulo-palpebral muscle of Hessar as the probable source of its histogenesis. Orbital leiomyomas constitute a spectrum of tumors ranging from vascular leiomyoma or angiomyoma with a large component of capillaries to a rather solid smooth muscle tumor that does not feature a conspicuous vascular component [2]. Because of the occurrence of vascular leiomyomas, the vascular smooth muscles in the vessel walls and the pericytes are also proposed as the source of origin of this tumor in the orbit [3]. In our case, the tumor extended intracranially through the superior orbital fissure; therefore, a vascular origin seems likely, because many of the ophthalmic veins pass through the superior orbital fissure. INTRACRANIAL EXTENSION AND RECURRENCE Jakobiec et al [4] reported a 34-year-old woman with an orbital leiomyoma that was partially excised through a lateral canthotomy, leaving behind the part that was adherent at the superior orbital fissure. Though widening of the superior orbital fissure was seen on X-ray, the extent of intracranial extension was not defined. The residue subsequently showed regrowth 7 years later and developed sarcomatous change 6 years after radiother-
330 Surg Neurol 2000;54:327–30
1
Kulkarni et al
Features of Some Reported Cases of Orbital Leiomyoma
LOCATION
INTRACRANIAL
AGE/SEX
AUTHOR/YEAR
IN ORBIT
EXTENSION
OF RESECTION
18/F 17/M 17/M 34/M
Nath & Shukla 1962 Jacobiec et al 1975 Jacobiec et al 1975 Jacobiec et al 1975
Apex Center Ant/med Apex
No No No Present
Total Partial Total Partial
5/M 39/M
Sanborn et al 1979 Betharia et al 1991
Sup. Ant.
No No
Total Total
apy. Nath et al [6] reported one case of orbital apex leiomyoma that was excised via a trans-orbital route. There was no intracranial extension. The natural history of these tumors in the orbit is not known. The location of the tumor in the orbit, extent of resection, and recurrence in some of the reported cases are shown in Table 1. No recurrence has been reported after total excision of an orbital leiomyoma; however, regrowth after partial excision has been reported in two cases [3,4]. In our case too, the tumor showed a significant increase in size, especially of the intracranial component, over a 4-year period after partial resection. Unlike previously reported cases, the tumor was adequately imaged, and was approached by a transcranial and lateral orbital route and was totally excised. The intracranial tumor was totally extradural, within the superior orbital fissure, but did not extend into the cavernous sinus. The tumor was found to be separated from nerves by a fascial plane, which enabled total excision without significant morbidity.
Conclusion Leiomyoma of the posterior orbit with intracranial extension is a very rare entity, and should be differentiated from other common tumors of the orbit like schwannoma and neurofibroma on histology. It can regrow after partial excision, is radioresistant, and may show sarcomatous change. Therefore, total excision by a transcranial route is advised. REFERENCES 1. Betharia SM, Arora R, Kishore K, Patil ND. Leiomyoma of the orbit. Ind J Ophthal 1991;39:35–7.
EXTENT
RECURRENCE No Progression after 6 yrs No Regrowth after 7 years, sarcomatous change after radiotherapy No No
2. Cherrik HM, Dunlap C, King O. Leiomyoma of the oral cavity. Review of literature and clinicopathologic study of seven new cases. Oral Surg 1973;35:54 –57. 3. Jacobiec FA, Jones IS, Tannenbaum M. Leiomyoma: an unusual tumor of the orbit. Br J Ophthalmol 1973;57: 825–31. 4. Jacobiec FA, Howard GM, Rosen M, Wolff M. Leiomyoma and leiomyosarcoma of the orbit. Am J Ophthalmol 1975;80:1028 – 42. 5. Meyer SL, Fine BS, Font RL, Zimmerman LE. Leiomyoma of the ciliary body: electron microscopic verification. Am J Ophthalmol 1968;66:1061– 68. 6. Nath K, Shukla BR. Orbital leiomyoma and its origin. Br J Ophthalmol 1963;47:369 –71. 7. Sanborn GE, Valenzuela RE, Green WR. Leiomyoma of the orbit. Am J Ophthalmol 1979;87:371–75.
COMMENTARY
Leiomyoma of the orbit is indeed a rare tumor. In our series of over 1,500 orbital tumors, we have not diagnosed or operated on one of these. It would certainly be easy to mistake this on neuroimaging for a neurofibroma or even a meningioma, but as the authors point out, the tumor was completely encapsulated and was removed in a fairly straightforward fashion, with preservation of vision. The authors emphasize that total excision should be done, if at all possible, at the first procedure, since—as demonstrated by this case—they do recur. This rare but curable tumor should be kept in mind when considering the differential diagnosis of orbital apex neoplasms. Joseph C. Maroon, M.D. Department of Neurosurgery University of Pittsburgh Medical Center Pittsburgh, Pennsylvania