Ophthal Plast Reconstr Surg, Vol. 30, No. 6, 2014
Extragnathic Sino-Orbital Myxoma: An Extremely Rare Tumor at an Even Rarer Site Mohammad Javed Ali, M.D., F.R.C.S.*, Debarati Saha, M.D.*, Geeta K. Vemuganti, M.D.†, and Milind N. Naik, M.D.* Abstract: Myxomas are rare benign tumors of primitive mesenchymal origin that usually arise from the soft tissues and occasionally from the bones. Bony myxomas predominantly arise from the jaw bone and hence are also known as gnathic myxomas. Any other bony myxomas are termed as extragnathic. In view of rarity, uncertain histogenesis, nonspecific clinical features, confusing terminology, and unclear management protocols, the backbone of diagnosis is a thorough histopathologic examination. Only 2 reports on myxomas arising from the frontal sinus have been documented previously. A young man presented with proptosis secondary to a sino-orbital mass lesion arising from the frontal sinus. The patient underwent complete excision biopsy and bone curettage. Following excision, the patient is symptom free at 6 months of follow up and showed no evidence of recurrence.
T
umors of primitive mesenchymal origin are rare. 1–10 Myxomas may be of soft tissue origin or bony origin. 1–10 Bony myxomas predominantly arise from the jaw bone and hence are also known as gnathic myxomas. Any other bony myxomas are termed as extragnathic. 2–5 Few cases of extragnathic myxoma have been reported in literature. Frontal sinus is one such rare site, and a sino-orbital extension presenting as a proptosis from this sinus is even rarer. Unlike gnathic myxomas, gender and age predilections are not clear for sino-orbital myxomas. To the best of authors’ knowledge, only 2 reports of frontal sinus myxomas have been documented but without any gross orbital extension. 3,4 The present case is unique because it predominantly invaded the orbit from the frontal sinus in an aggressive fashion. In view of rarity, uncertain histogenesis, nonspecific clinical features, and confusing terminology, the backbone of diagnosis is a thorough histopathologic examination.
CASE REPORT A 20-year-old man presented with rapid but painless protrusion of his left eye of 1-month duration (Fig. 1A). He had no visual complaints. Ophthalmic examination revealed his best-corrected visual acuity in OU to be 20/20, and color vision was normal. Hertel exophthalmometry showed an 8-mm abaxial proptosis and hypoglobus of OS (Fig. 1A). Ocular motility was restricted in upgaze and lateral gazes.
*Dacryology Service and †Ocular Pathology Service, L.V. Prasad Eye Institute, Hyderabad, Andhra Pradesh, India Accepted for publication August 1, 2013. The authors disclose no financial or conflicts of interest. Address correspondence and reprint requests to Mohammad Javed Ali, M.D., F.R.C.S., Ophthalmic Plastics and Ocular Oncology, L.V. Prasad Eye Institute, Road No 2, Banjara Hills, Hyderabad, Andhra Pradesh 500028, India. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000002
Case Reports
Valsalva was negative, and a soft mass could be palpated deep in the superior orbit. CT scan showed a well-defined, heterodense mass lesion (44 × 22 mm) in left superior orbit extending from anterior orbit up to the apex posteriorly (Fig. 1B) and medially up to medial orbital wall (Fig. 1C), displacing the globe slightly downward. The lesion appeared as multiloculated, separated by thin bony trabeculae, arising from frontal sinus, conical in shape giving a classical appearance of “soap-bubble or tennis-racket pattern” (Fig. 1D). The roof and the medial wall showed significant thinning but no breach (Fig. 1B). A diagnostic incision biopsy was planned and performed through subbrow and extraperiosteal approach. Intraoperatively, a soft-to-firm mass was palpable, and an incision over the lesion led to copious ooze of sticky and gelatinous material. The entire lesion was subsequently curetted. Numerous bony septae were carefully removed along with the lining of subsequent empty cavity. The patient is currently on a close follow up, and there has been no recurrence at 6 months postoperatively. Histopathology. Gross examination showed multiple grayish white soft and flat bony tissues without any evidence of surface hemorrhages or necrosis. Microscopic examination showed a paucicellular tumor with few areas of bony trabeculae and large areas of myxoid tissue (Fig. 2A). There was abundance of myxoid tissues all over. The myxoid areas consisted of loose and scattered spindle and stellate cells with mildly enlarged nucleus and moderate cytoplasm but without any mitotic figure (Fig. 2B). Few reticulin fibers were found within the mucoid stroma (Fig. 2B). The bony trabeculae were lined by osteoblasts (Fig. 2A), and few areas of osteoid-like tissue (Fig. 2C) and occasional osteoclasts (Fig. 2D) were also noted. Alcian blue stain demarcated myxoid area suggestive of stroma rich in hyaluronic acid (Fig. 2E). Immunohistochemistry showed positive staining for vimentin (Fig. 2F) and negative for cytokeratin. These features were consistent with the diagnosis of extragnathic sino-orbital myxoma.
DISCUSSION Myxoma occurs in both bone and soft tissue.1–10 Although intraosseous myxoma has been reported in long bones, many of the head and neck myxomas occur in the mandible, followed by the maxilla, therefore, the terms “gnathic or odontogenic myxoma” are often used.2 At present, its histogenesis is uncertain, but 2 possibilities of origin are primitive odontogenic mesenchyme and altered fibroblasts. Unlike gnathic myxomas, gender and age predilections are not clear for sino-orbital myxomas.1–4 Extragnathic tumors are uncommon and primarily involve the sino-nasal tract; specifically, the maxillary sinus (antrum) is most often involved with secondary extension in the nasal cavity. Chen et al.5 and Ducic et al.6 reported a case each of odontogenic myxoma arising from the maxillary sinus and invading the orbit walls. Two cases of orbital soft tissue myxomas demonstrated overlying bony changes and were excised en bloc. Hidayat et al.1 studied 6 cases of soft tissue myxomas of the orbit and 1 case of a supero-lateral mass with overlying bone destruction, recurred following an excision and was treated by removal of the lateral orbital roof. Candy et al.7 also reported a similar case of a supero-lateral orbital soft tissue myxoma with overlying erosion of lateral wall and sphenoid bone.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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Ophthal Plast Reconstr Surg, Vol. 30, No. 6, 2014
Case Reports
FIG. 1. Clinical photograph showing a gross abaxial proptosis of the OS (A). CT scan, axial cut, showing a large heterogeneous mass lesion extending up to the apex (B). CT scan, coronal cut, showing the lesion invading the orbit with numerous trabeculae and thinning of the orbital roof (C). CT scan, coronal cut, bone window, showing the classical “soap-bubble” or “tennis-racket” appearance (D).
Radiologically, CT scans may show a characteristic “soap-bubble” or “honey comb” appearance caused by a multiloculated lesion separated by bony trabeculae from the periphery.5 Expansion of orbit and overlying bony changes such as thinning and erosions may be noted. MRI findings reported for myxomas arising from frontal sinuses include hypointense signals on T1-weighted and hyperintense on T2-weighted images.3 Stout8 in 1948 reported histopathologic details of myxomas and found them to be composed of scattered stellate cells in a loose mucoid stroma through which reticulin fibers course in various directions. Alcian blue staining can demarcate the mucoid areas.9 Histopathologic differential diagnosis can be tumors of the orbit with myxoid degenerations, such as schwannoma, malignant fibrous histiocytoma, and chondrosarcoma. The diagnosis can be established based on cellular morphology. Immunohistochemistry aids in the diagnosis, where most of the myxomas stain positively for vimentin and negatively for neural markers such as neuronspecific enolase and glial fibrillary acidic protein and for epithelial markers such as cytokeratin, confirming its mesodermal origin. Electron microscopic features show 2 different types of cells. Round or irregular cells have a large nucleus with abundant ribosomes and rough endoplasmic reticulum, whereas stellate cells with elongated mitochondria and filaments arranged parallel to the long axis of cells.10 It is also known that extragnathic myxoma-like features may result in
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myxoid degeneration of bone tumors, such as fibrosarcoma and chondrosarcoma.1 Soft tissue myxomas are known to have systemic associations, such as Carney complex, Mazabraud syndrome, or McCune Albright syndrome1; however, such associations have not been found with sino-orbital myxomas, more specifically those arising from the frontal sinus. It is important to understand that lack of reporting of systemic associations may also be due to exceptional rarity of the lesion. Although slow-growing, bony myxomas may behave in an aggressive fashion clinically. Metastasis does not occur; however, recurrence rates following excision are reported to be as high as 25%, usually within 2 years following excision but may be delayed as well.1–10 Hence, a total en bloc surgical resection with wide margins is recommended for managing these tumors. A point of clinical importance for oculoplastic surgeons is that if ruptured during the surgery, the contents may remarkably appear like vitreous and lack of awareness of this fact may raise an undue alarm of globe injury! External beam radiotherapy, although described in one case of benign orbital myxoma with infiltrative pattern on histology, is not a well-established treatment modality.10 Despite high recurrence, the prognosis is considered good, depending on the anatomical location.1–10 A close follow up for 2 years and a regular follow up for 5 years have been recommended in odontogenic myxomas to declare a patient disease free.5
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 30, No. 6, 2014
Case Reports
Severe Neutropenia Presenting With Candida Albicans Eyelid Abscess in a 1-Year-Old Child Bryan R. Costin, M.D.*, Carrie E. Costin, M.D.†, Palak B. Wall, M.D.*, Jedediah I. McClintic, M.D.*, Charles B. Foster, M.D.‡, Lisa R. Hackney, M.D.‡, Elias I. Traboulsi, M.D.*, and Julian D. Perry, M.D.* Abstract: A previously healthy 1-year-old boy presented with a right lower eyelid abscess. Oral and topical antibiotics were initiated, and the lesion was drained and explored. Despite medical and surgical treatment, the abscess failed to resolve completely and formed again 1 month later. A second incision and drainage procedure was performed, and Candida albicans grew in cultures. Additional workup revealed severe neutropenia with an absolute neutrophil count of 0.18 k/μl (1.19–7.21 k/μl). The abscess resolved after 2 weeks of oral fluconazole. C. albicans eyelid abscess may be the presenting sign of systemic immunodeficiency.
FIG. 2. Microphotographs showing few areas of bony trabeculae and large areas of myxoid tissue (hematoxylineosin, ×100; A), paucicellular tumor with stellate cells and intervening reticulin fibers (hematoxylin-eosin, ×400; B), osteoid-like areas (hematoxylin-eosin, ×100; C), occasional osteoclasts (hematoxylin-eosin, ×400; D), and positive staining for acid mucopolysaccharide (Alcian blue, ×100; E). Immunohistochemical staining showing strong positivity to vimentin reflecting the mesenchymal origin (vimentin, ×400; F).
In conclusion, extragnathic sino-orbital myxomas are rare and should be considered in differential diagnosis of atypical sinoorbital lesion. Although these are low-grade benign neoplasm, a complete curettage or en bloc surgical excision is the treatment of choice. A high recurrence rate warrants a close follow up.
REFERENCES 1. Hidayat AA, Flint A, Marentette L, et al. Myxomas and angiomyxomas of the orbit: a clinicopathologic study of 6 cases. Ophthalmology 2007;114:1012–9. 2. Noffke CE, Raubenheimer EJ, Chabikuli NJ, et al. Odontogenic myxoma: review of the literature and report of 30 cases from South Africa. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:101–9. 3. Cincik H, Ertugrul E, Gungor A, et al. Pathology quiz case 2. Myxoma of the frontal sinus. Arch Otolaryngol Head Neck Surg 2007;133:412, 415. 4. Gryczyński M. Myxomas of the frontal sinus. Pol Tyg Lek 1972;27:1315–7. 5. Chen HH, Streubel SO, Durairaj VD. Odontogenic myxoma with orbital involvement. Ophthal Plast Reconstr Surg 2013;29:e47–9. 6. Ducic I, Davison SP, Woll S, et al. Maxillary infraorbital myxoma: reconstruction with vascularized temporal bone. Otolaryngol Head Neck Surg 2003;128:426–7. 7. Candy EJ, Miller NR, Carson BS. Myxoma of bone involving the orbit. Arch Ophthalmol 1991;109:919–20. 8. Stout AP. Myxoma, the tumor of primitive mesenchyme. Ann Surg 1948;127:706–19. 9. Rambhatla S, Subramanian N, Gangadhara Sundar JK, et al. Myxoma of the orbit. Indian J Ophthalmol 2003;51:85–7. 10. Lieb WE, Goebel HH, Wallenfang T. Myxoma of the orbit: a clinicopathologic report. Graefes Arch Clin Exp Ophthalmol 1990;228:28–32.
C
andida albicans is an organism responsible for a wide variety of infectious processes. In the pediatric population, this fungus most commonly causes a superficial dermatitis such as diaper rash and mucous membrane infections such as thrush.1 Candida infections are more commonly seen in the immunocompromised in general and in neutropenics in particular.2,3 Cellular functions at the molecular level may explain the virulence of this organism in neutropenia.4 However, C. albicans rarely causes skin abscesses even in immunocompromised children and adults.5–7 A PubMed search performed in the English language in May 2013 did not reveal any reports of C. albicans eyelid abscess. Factors predisposing to cutaneous Candida abscesses include diabetes mellitus, immunodeficiency, malignancy, retained foreign body, traumatic inoculation, use of broad spectrum antibiotics, and candidemia.8 The authors report a case of C. albicans eyelid abscess as the presenting feature of severe neutropenia. All clinical photographs were obtained after written consent was obtained from the patient’s guardian in accordance with Health Insurance Portability and Accountability Act.
CASE REPORT A 1-year-old boy presented to the pediatric ophthalmology service with a 2-month history of a recurrent right lower eyelid abscess that had a fluctuating course in which it would develop, drain spontaneously, improve without complete resolution, and then develop again. The patient’s mother was not aware of previous trauma at this location. The child was born at 33 weeks after an uncomplicated pregnancy. He spent 2 weeks in the neonatal intensive care unit for prematurity and low birth weight, but had no subsequent growth or developmental delay. He had no other significant medical history, and there had been no other skin lesions.
*Cole Eye Institute, Cleveland Clinic; †Center for Human Genetics, University Hospitals Case Medical Center; and ‡Cleveland Clinic Children’s Hospital, Cleveland, Ohio, U.S.A. Accepted for publication August 4, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Bryan R. Costin, M.D., Cole Eye Institute, Cleveland Clinic, 9500 Euclid Ave., Cleveland, OH 44195. E-mail: [email protected] DOI: IOP.0000000000000003
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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Extragnathic Sino-Orbital Myxoma: An Extremely Rare Tumor at an Even Rarer Site Mohammad Javed Ali, M.D., F.R.C.S.*, Debarati Saha, M.D.*, Geeta K. Vemuganti, M.D.†, and Milind N. Naik, M.D.* Abstract: Myxomas are rare benign tumors of primitive mesenchymal origin that usually arise from the soft tissues and occasionally from the bones. Bony myxomas predominantly arise from the jaw bone and hence are also known as gnathic myxomas. Any other bony myxomas are termed as extragnathic. In view of rarity, uncertain histogenesis, nonspecific clinical features, confusing terminology, and unclear management protocols, the backbone of diagnosis is a thorough histopathologic examination. Only 2 reports on myxomas arising from the frontal sinus have been documented previously. A young man presented with proptosis secondary to a sino-orbital mass lesion arising from the frontal sinus. The patient underwent complete excision biopsy and bone curettage. Following excision, the patient is symptom free at 6 months of follow up and showed no evidence of recurrence.
T
umors of primitive mesenchymal origin are rare. 1–10 Myxomas may be of soft tissue origin or bony origin. 1–10 Bony myxomas predominantly arise from the jaw bone and hence are also known as gnathic myxomas. Any other bony myxomas are termed as extragnathic. 2–5 Few cases of extragnathic myxoma have been reported in literature. Frontal sinus is one such rare site, and a sino-orbital extension presenting as a proptosis from this sinus is even rarer. Unlike gnathic myxomas, gender and age predilections are not clear for sino-orbital myxomas. To the best of authors’ knowledge, only 2 reports of frontal sinus myxomas have been documented but without any gross orbital extension. 3,4 The present case is unique because it predominantly invaded the orbit from the frontal sinus in an aggressive fashion. In view of rarity, uncertain histogenesis, nonspecific clinical features, and confusing terminology, the backbone of diagnosis is a thorough histopathologic examination.
CASE REPORT A 20-year-old man presented with rapid but painless protrusion of his left eye of 1-month duration (Fig. 1A). He had no visual complaints. Ophthalmic examination revealed his best-corrected visual acuity in OU to be 20/20, and color vision was normal. Hertel exophthalmometry showed an 8-mm abaxial proptosis and hypoglobus of OS (Fig. 1A). Ocular motility was restricted in upgaze and lateral gazes.
*Dacryology Service and †Ocular Pathology Service, L.V. Prasad Eye Institute, Hyderabad, Andhra Pradesh, India Accepted for publication August 1, 2013. The authors disclose no financial or conflicts of interest. Address correspondence and reprint requests to Mohammad Javed Ali, M.D., F.R.C.S., Ophthalmic Plastics and Ocular Oncology, L.V. Prasad Eye Institute, Road No 2, Banjara Hills, Hyderabad, Andhra Pradesh 500028, India. E-mail: [email protected] DOI: 10.1097/IOP.0000000000000002
Case Reports
Valsalva was negative, and a soft mass could be palpated deep in the superior orbit. CT scan showed a well-defined, heterodense mass lesion (44 × 22 mm) in left superior orbit extending from anterior orbit up to the apex posteriorly (Fig. 1B) and medially up to medial orbital wall (Fig. 1C), displacing the globe slightly downward. The lesion appeared as multiloculated, separated by thin bony trabeculae, arising from frontal sinus, conical in shape giving a classical appearance of “soap-bubble or tennis-racket pattern” (Fig. 1D). The roof and the medial wall showed significant thinning but no breach (Fig. 1B). A diagnostic incision biopsy was planned and performed through subbrow and extraperiosteal approach. Intraoperatively, a soft-to-firm mass was palpable, and an incision over the lesion led to copious ooze of sticky and gelatinous material. The entire lesion was subsequently curetted. Numerous bony septae were carefully removed along with the lining of subsequent empty cavity. The patient is currently on a close follow up, and there has been no recurrence at 6 months postoperatively. Histopathology. Gross examination showed multiple grayish white soft and flat bony tissues without any evidence of surface hemorrhages or necrosis. Microscopic examination showed a paucicellular tumor with few areas of bony trabeculae and large areas of myxoid tissue (Fig. 2A). There was abundance of myxoid tissues all over. The myxoid areas consisted of loose and scattered spindle and stellate cells with mildly enlarged nucleus and moderate cytoplasm but without any mitotic figure (Fig. 2B). Few reticulin fibers were found within the mucoid stroma (Fig. 2B). The bony trabeculae were lined by osteoblasts (Fig. 2A), and few areas of osteoid-like tissue (Fig. 2C) and occasional osteoclasts (Fig. 2D) were also noted. Alcian blue stain demarcated myxoid area suggestive of stroma rich in hyaluronic acid (Fig. 2E). Immunohistochemistry showed positive staining for vimentin (Fig. 2F) and negative for cytokeratin. These features were consistent with the diagnosis of extragnathic sino-orbital myxoma.
DISCUSSION Myxoma occurs in both bone and soft tissue.1–10 Although intraosseous myxoma has been reported in long bones, many of the head and neck myxomas occur in the mandible, followed by the maxilla, therefore, the terms “gnathic or odontogenic myxoma” are often used.2 At present, its histogenesis is uncertain, but 2 possibilities of origin are primitive odontogenic mesenchyme and altered fibroblasts. Unlike gnathic myxomas, gender and age predilections are not clear for sino-orbital myxomas.1–4 Extragnathic tumors are uncommon and primarily involve the sino-nasal tract; specifically, the maxillary sinus (antrum) is most often involved with secondary extension in the nasal cavity. Chen et al.5 and Ducic et al.6 reported a case each of odontogenic myxoma arising from the maxillary sinus and invading the orbit walls. Two cases of orbital soft tissue myxomas demonstrated overlying bony changes and were excised en bloc. Hidayat et al.1 studied 6 cases of soft tissue myxomas of the orbit and 1 case of a supero-lateral mass with overlying bone destruction, recurred following an excision and was treated by removal of the lateral orbital roof. Candy et al.7 also reported a similar case of a supero-lateral orbital soft tissue myxoma with overlying erosion of lateral wall and sphenoid bone.
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
e157
Ophthal Plast Reconstr Surg, Vol. 30, No. 6, 2014
Case Reports
FIG. 1. Clinical photograph showing a gross abaxial proptosis of the OS (A). CT scan, axial cut, showing a large heterogeneous mass lesion extending up to the apex (B). CT scan, coronal cut, showing the lesion invading the orbit with numerous trabeculae and thinning of the orbital roof (C). CT scan, coronal cut, bone window, showing the classical “soap-bubble” or “tennis-racket” appearance (D).
Radiologically, CT scans may show a characteristic “soap-bubble” or “honey comb” appearance caused by a multiloculated lesion separated by bony trabeculae from the periphery.5 Expansion of orbit and overlying bony changes such as thinning and erosions may be noted. MRI findings reported for myxomas arising from frontal sinuses include hypointense signals on T1-weighted and hyperintense on T2-weighted images.3 Stout8 in 1948 reported histopathologic details of myxomas and found them to be composed of scattered stellate cells in a loose mucoid stroma through which reticulin fibers course in various directions. Alcian blue staining can demarcate the mucoid areas.9 Histopathologic differential diagnosis can be tumors of the orbit with myxoid degenerations, such as schwannoma, malignant fibrous histiocytoma, and chondrosarcoma. The diagnosis can be established based on cellular morphology. Immunohistochemistry aids in the diagnosis, where most of the myxomas stain positively for vimentin and negatively for neural markers such as neuronspecific enolase and glial fibrillary acidic protein and for epithelial markers such as cytokeratin, confirming its mesodermal origin. Electron microscopic features show 2 different types of cells. Round or irregular cells have a large nucleus with abundant ribosomes and rough endoplasmic reticulum, whereas stellate cells with elongated mitochondria and filaments arranged parallel to the long axis of cells.10 It is also known that extragnathic myxoma-like features may result in
e158
myxoid degeneration of bone tumors, such as fibrosarcoma and chondrosarcoma.1 Soft tissue myxomas are known to have systemic associations, such as Carney complex, Mazabraud syndrome, or McCune Albright syndrome1; however, such associations have not been found with sino-orbital myxomas, more specifically those arising from the frontal sinus. It is important to understand that lack of reporting of systemic associations may also be due to exceptional rarity of the lesion. Although slow-growing, bony myxomas may behave in an aggressive fashion clinically. Metastasis does not occur; however, recurrence rates following excision are reported to be as high as 25%, usually within 2 years following excision but may be delayed as well.1–10 Hence, a total en bloc surgical resection with wide margins is recommended for managing these tumors. A point of clinical importance for oculoplastic surgeons is that if ruptured during the surgery, the contents may remarkably appear like vitreous and lack of awareness of this fact may raise an undue alarm of globe injury! External beam radiotherapy, although described in one case of benign orbital myxoma with infiltrative pattern on histology, is not a well-established treatment modality.10 Despite high recurrence, the prognosis is considered good, depending on the anatomical location.1–10 A close follow up for 2 years and a regular follow up for 5 years have been recommended in odontogenic myxomas to declare a patient disease free.5
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
Ophthal Plast Reconstr Surg, Vol. 30, No. 6, 2014
Case Reports
Severe Neutropenia Presenting With Candida Albicans Eyelid Abscess in a 1-Year-Old Child Bryan R. Costin, M.D.*, Carrie E. Costin, M.D.†, Palak B. Wall, M.D.*, Jedediah I. McClintic, M.D.*, Charles B. Foster, M.D.‡, Lisa R. Hackney, M.D.‡, Elias I. Traboulsi, M.D.*, and Julian D. Perry, M.D.* Abstract: A previously healthy 1-year-old boy presented with a right lower eyelid abscess. Oral and topical antibiotics were initiated, and the lesion was drained and explored. Despite medical and surgical treatment, the abscess failed to resolve completely and formed again 1 month later. A second incision and drainage procedure was performed, and Candida albicans grew in cultures. Additional workup revealed severe neutropenia with an absolute neutrophil count of 0.18 k/μl (1.19–7.21 k/μl). The abscess resolved after 2 weeks of oral fluconazole. C. albicans eyelid abscess may be the presenting sign of systemic immunodeficiency.
FIG. 2. Microphotographs showing few areas of bony trabeculae and large areas of myxoid tissue (hematoxylineosin, ×100; A), paucicellular tumor with stellate cells and intervening reticulin fibers (hematoxylin-eosin, ×400; B), osteoid-like areas (hematoxylin-eosin, ×100; C), occasional osteoclasts (hematoxylin-eosin, ×400; D), and positive staining for acid mucopolysaccharide (Alcian blue, ×100; E). Immunohistochemical staining showing strong positivity to vimentin reflecting the mesenchymal origin (vimentin, ×400; F).
In conclusion, extragnathic sino-orbital myxomas are rare and should be considered in differential diagnosis of atypical sinoorbital lesion. Although these are low-grade benign neoplasm, a complete curettage or en bloc surgical excision is the treatment of choice. A high recurrence rate warrants a close follow up.
REFERENCES 1. Hidayat AA, Flint A, Marentette L, et al. Myxomas and angiomyxomas of the orbit: a clinicopathologic study of 6 cases. Ophthalmology 2007;114:1012–9. 2. Noffke CE, Raubenheimer EJ, Chabikuli NJ, et al. Odontogenic myxoma: review of the literature and report of 30 cases from South Africa. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:101–9. 3. Cincik H, Ertugrul E, Gungor A, et al. Pathology quiz case 2. Myxoma of the frontal sinus. Arch Otolaryngol Head Neck Surg 2007;133:412, 415. 4. Gryczyński M. Myxomas of the frontal sinus. Pol Tyg Lek 1972;27:1315–7. 5. Chen HH, Streubel SO, Durairaj VD. Odontogenic myxoma with orbital involvement. Ophthal Plast Reconstr Surg 2013;29:e47–9. 6. Ducic I, Davison SP, Woll S, et al. Maxillary infraorbital myxoma: reconstruction with vascularized temporal bone. Otolaryngol Head Neck Surg 2003;128:426–7. 7. Candy EJ, Miller NR, Carson BS. Myxoma of bone involving the orbit. Arch Ophthalmol 1991;109:919–20. 8. Stout AP. Myxoma, the tumor of primitive mesenchyme. Ann Surg 1948;127:706–19. 9. Rambhatla S, Subramanian N, Gangadhara Sundar JK, et al. Myxoma of the orbit. Indian J Ophthalmol 2003;51:85–7. 10. Lieb WE, Goebel HH, Wallenfang T. Myxoma of the orbit: a clinicopathologic report. Graefes Arch Clin Exp Ophthalmol 1990;228:28–32.
C
andida albicans is an organism responsible for a wide variety of infectious processes. In the pediatric population, this fungus most commonly causes a superficial dermatitis such as diaper rash and mucous membrane infections such as thrush.1 Candida infections are more commonly seen in the immunocompromised in general and in neutropenics in particular.2,3 Cellular functions at the molecular level may explain the virulence of this organism in neutropenia.4 However, C. albicans rarely causes skin abscesses even in immunocompromised children and adults.5–7 A PubMed search performed in the English language in May 2013 did not reveal any reports of C. albicans eyelid abscess. Factors predisposing to cutaneous Candida abscesses include diabetes mellitus, immunodeficiency, malignancy, retained foreign body, traumatic inoculation, use of broad spectrum antibiotics, and candidemia.8 The authors report a case of C. albicans eyelid abscess as the presenting feature of severe neutropenia. All clinical photographs were obtained after written consent was obtained from the patient’s guardian in accordance with Health Insurance Portability and Accountability Act.
CASE REPORT A 1-year-old boy presented to the pediatric ophthalmology service with a 2-month history of a recurrent right lower eyelid abscess that had a fluctuating course in which it would develop, drain spontaneously, improve without complete resolution, and then develop again. The patient’s mother was not aware of previous trauma at this location. The child was born at 33 weeks after an uncomplicated pregnancy. He spent 2 weeks in the neonatal intensive care unit for prematurity and low birth weight, but had no subsequent growth or developmental delay. He had no other significant medical history, and there had been no other skin lesions.
*Cole Eye Institute, Cleveland Clinic; †Center for Human Genetics, University Hospitals Case Medical Center; and ‡Cleveland Clinic Children’s Hospital, Cleveland, Ohio, U.S.A. Accepted for publication August 4, 2013. The authors have no financial or conflicts of interest to disclose. Address correspondence and reprint requests to Bryan R. Costin, M.D., Cole Eye Institute, Cleveland Clinic, 9500 Euclid Ave., Cleveland, OH 44195. E-mail: [email protected] DOI: IOP.0000000000000003
© 2014 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.
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