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Thymic Carcinoma Metastatic to the Orbit
Thymic Carcinoma Metastatic to the Orbit Frank A. Stockl, MD, Nancy Tucker, MD, and Miguel N. Burnier, MD, PhD PURPOSE: To report a case of small cell carcinoma of the thymus metastatic to the orbit. METHODS: At age 37 years, a man who had been previously diagnosed and treated for small cell carcinoma of the thymus was initially examined for vertical diplopia and left proptosis. Computed tomographic scan disclosed a soft tissue mass in the superior aspect of the left orbit. A fine-needle aspiration biopsy of the mass was performed and submitted for cytopathologic examination. RESULT: The cytopathology and immunopathology disclosed malignant cells consistent with meta static small cell thymic carcinoma. CONCLUSION: This is the first reported case of a primary thymus gland tumor metastatic to the orbit. ETASTATIC TUMORS OF THE ORBIT MOST COMmonly originate from breast and lung.1 Other primary sites have been reported.1'3 To our knowledge, there are no reported cases of a primary tumor of the thymus gland metastasizing to the orbit. We describe a case of thymic carcinoma metastatic to the orbit. At age 37 years, our patient was initially examined by his family physician in June 1993 for cough and upper chest pain. A chest x-ray disclosed a mediastinal mass. A computed tomographic scan of the chest demonstrated a large soft tissue mass in the anterior mediastinum extending from the right peritracheal area to the ascending aorta at the level of the sternoclavicular joint. Mediastinal lymph nodes were also noted to be enlarged. The patient underwent flexible bronchoscopy, and a sternotomy with incisional biopsy of the mediastinal mass was performed. The biopsy of the mass disclosed a small cell carcino ma of the thymus. The patient's postoperative course was complicated Accepted for publication April 2, 1997. Oculoplastics and Orbital Service (F.A.S., N.T.) and Ophthalmic Pathology (M.N.B.), McGill University. Inquiries to Nancy Tucker, MD, Department of Ophthalmology, Royal Victoria Hospital, E4.54, 687 Pine Ave W, Montreal, Quebec, Canada H3A 1A1; e-mail: [email protected]
VOL.124, No. 3
FIGURE 1. Contrast-enhanced coronal computed tomo graphic scan of the orbits discloses an orbital soft tissue mass in the superior aspect of the left orbit (arrow).
by global aphasia and right hemiparesis. Computed tomographic scan of the brain showed an ischemic infarct of the left frontotemporal lobe area with no evidence of cerebral metastasis. The patient's symp toms gradually resolved over 1 week. The patient was subsequently treated with two cycles of cyclophosphamide, doxorubicin hydrochloride, and vincristine. Follow-up computed tomographic scan of the chest showed a decrease in the size of the mediastinal mass, but extensive mediastinal lymphadenopathy re mained. The patient then received radiation therapy with concomitant vincristine, procarbazine, and cisplatin. He did well until October 1994, when he was admitted with pneumonia and superior vena cava obstruction associated with recurrence of the medias tinal mass. Further treatment was declined by the patient. In February 1995, the patient was referred to the Oculoplastics and Orbital Service at McGill Univer sity because of left proptosis and vertical diplopia. Examination disclosed a visual acuity of RE, 20/15 and LE, 20/20. Pupils were equal and reactive, with no relative afferent pupillary defect. Extraocular move ments were painless but restricted in upgaze in the left eye. Hertel exophthalmometry disclosed 2 mm of proptosis of the left globe. Slit-lamp and fundus examinations were normal. A computed tomographic scan of the orbits dis closed a soft tissue mass measuring 1.5 cm in the superior aspect of the left orbit (Figure 1). A fineneedle aspiration biopsy of the orbital mass was
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«JRw*4ft$ FIGURE 2. (Left) Histopathologic section of fine-needle aspiration biopsy discloses small, poorly differentiated cells with scant cytoplasm, hyperchromic nuclei, and inconspicuous nucleoli with surrounding red blood cells (hematoxylin and eosin, X300). (Right) Histopathologic examination of the mediastinal mass biopsy demonstrates a uniform proliferation of small, round to oval cells with a high nuclear to cytoplasmic ratio. The chromatin is stippled with small nucleoli. In addition, several mitotic figures are present (hematoxylin and eosin, X400).
performed to secure a diagnosis. The cytopathology disclosed a uniform population of cells with scanty cytoplasm with small, hyperchromic, round nuclei (Figure 2, left). Immunohistochemistry was positive for cytokeratin and chromogranin and negative for leukocyte common antigen, which was consistent with a diagnosis of small cell thymic carcinoma. The morphologic characteristics of the cells obtained on fine-needle aspiration biopsy were identical to those obtained from the mediastinal incisional biopsy that was performed in 1993, confirming the clinical sus picion of thymic carcinoma metastatic to the orbit (Figure 2, left). Palliative treatment with external beam radiation to the left orbit resulted in complete resolution of the patient's ocular signs and symptoms. However, the patient continued to feel unwell, with weakness and shortness of breath. Subsequently, he returned to his homeland in Singapore to seek alternative therapies and has been lost to follow-up. Thymic tumors are the most common neoplasms in the anterior mediastinum of adults.4 Derived from thymic epithelial cells, the majority of tumors are well circumscribed and encapsulated and do not 402
invade the surrounding structures. Thymic tumors are divided into two categories: thymomas and thymic carcinomas. Thymomas are classified as benign or malignant based not on cellular features but rather on evidence of macroscopic invasion at the time of surgery. Thymomas rarely metastasize, but when this does occur, the most frequent sites of involvement are bone, lymph nodes, and liver. Rarely, thymic epitheli al neoplasms display obvious cytologic malignant features and are then termed "thymic carcinoma."5 The mortality rate of thymic carcinoma is much higher than that of thymoma, and a clear separation of these two entities is therefore justified.5 This case demonstrates the usefulness of fine-needle aspiration biopsy in confirming a suspected clinical diagnosis with minimal patient discomfort and morbidity. Rare tumors can be confidently diagnosed with fine-needle aspiration biopsy with minimal tissue, particularly in cases in which the biopsy of the primary tumor has already been obtained. REFERENCES 1. Shields CL, Shields JA, Peggs M. Tumors metastatic to the orbit. Ophthalmic Plast Reconstr Surg 1988;4:73-80.
AMERICAN JOURNAL OF OPHTHALMOLOGY
SEPTEMBER 1997
2. Freedman MI, Folk JC. Metastatic tumors to the eye and orbit: patient survival and clinical characteristics. Arch Ophthalmol 1987;105:1215-1219. 3. Font RL, Ferry AP. Carcinoma metastatic to the eye and orbit, III: a clinicopathological study of 28 cases metastatic to the orbit. Cancer 1976;38:1326-1335. 4. Tomiak EM, Evans WK. The role of chemotherapy in invasive thymoma: a review of the literature and consideration for future clinical trials. Crit Rev Oncol Hematol 1993; 15: 113-124. 5. Troung LD, Mody DR, Cagle PT, Jackson-York GL, Schwartz MR, Wheeler TM. Thymic carcinoma: a clinicopathologic study of 13 cases. Am J Surg Pathol 1990;14:151-166.
Acquired Capillary Hemangioma of the Eyelid in an Adult Brent A. Murphy, MD, Gamil S. Dawood, MD, and Curtis E. Margo, MD
FIGURE 1. A 7-mm blue nodule (arrows) is present in the outer portion of the right lower eyelid. The lesion had a rubbery consistency.
PURPOSE: To report a case of an acquired capil lary hemangioma of the eyelid in an adult. METHODS: A 38-year-old man developed a dis crete blue nodular mass in the lower eyelid. The lesion grew slowly for 6 months and then remained stable in size. Excisional biopsy was performed. RESULT: Histopathologic examination of an exci sional biopsy specimen of the nodular mass dis closed a capillary hemangioma. CONCLUSIONS: Capillary hemangioma of the eyelid can occur in adults but should be distin guished from other acquired vascular lesions, in cluding angiosarcoma.
C
APILLARY HEMANGIOMA IS THE MOST COMMON congenital vascular tumor of the periorbital re gion.1 Most capillary hemangiomas appear at birth or . within the first weeks of life. They often display exuberant growth over the next 6 to 12 months and may result in occlusion of the visual axis or anisometropic amblyopia. This growth phase is followed by spontaneous regression during the first decade of life. Capillary hemangioma has not been recognized as an acquired periorbital lesion in adults.
Accepted for publication April 28, 1997. Departments of Ophthalmology (B.A.M.) and Medicine (G.S.D.), University of South Alabama, and Department of Ophthalmology (C.E.M.), University of South Florida. Inquiries to Brent A. Murphy, MD, P.O. Box 8448, Mobile, AL J6689-0448. VOL.124, No. 3
FIGURE 2. Capillary hemangioma showing closely packed vascular spaces lined by uniform endothelial cells and scant extracellular matrix (hematoxylin and eosin, X390). (Inset) Low magnification of biopsy specimen showing the cellularity and well-circumscribed border of the vascular tumor (hematoxylin and eosin, X16).
A 38-year-old m a n was referred for evaluation of a
lower eyelid tumor. The lesion was first noted 1 year earlier as a small dark spot in the eyelid. The lesion had enlarged for 6 months and then remained stable in size. Increased vascularity of the overlying skin and blue discoloration had developed during the ensuing months. There were no associated ophthalmic symp toms, other cutaneous lesions, or history of anteced ent trauma. External examination showed a blue subcutaneous nodule in the right lower eyelid with
BRIEF REPORTS
403
VOL.124, No. 3
FIGURE 1. Contrast-enhanced coronal computed tomo graphic scan of the orbits discloses an orbital soft tissue mass in the superior aspect of the left orbit (arrow).
by global aphasia and right hemiparesis. Computed tomographic scan of the brain showed an ischemic infarct of the left frontotemporal lobe area with no evidence of cerebral metastasis. The patient's symp toms gradually resolved over 1 week. The patient was subsequently treated with two cycles of cyclophosphamide, doxorubicin hydrochloride, and vincristine. Follow-up computed tomographic scan of the chest showed a decrease in the size of the mediastinal mass, but extensive mediastinal lymphadenopathy re mained. The patient then received radiation therapy with concomitant vincristine, procarbazine, and cisplatin. He did well until October 1994, when he was admitted with pneumonia and superior vena cava obstruction associated with recurrence of the medias tinal mass. Further treatment was declined by the patient. In February 1995, the patient was referred to the Oculoplastics and Orbital Service at McGill Univer sity because of left proptosis and vertical diplopia. Examination disclosed a visual acuity of RE, 20/15 and LE, 20/20. Pupils were equal and reactive, with no relative afferent pupillary defect. Extraocular move ments were painless but restricted in upgaze in the left eye. Hertel exophthalmometry disclosed 2 mm of proptosis of the left globe. Slit-lamp and fundus examinations were normal. A computed tomographic scan of the orbits dis closed a soft tissue mass measuring 1.5 cm in the superior aspect of the left orbit (Figure 1). A fineneedle aspiration biopsy of the orbital mass was
BRIEF REPORTS
401
;€& JS^*
.
•
*
&
**#
■
*
«JRw*4ft$ FIGURE 2. (Left) Histopathologic section of fine-needle aspiration biopsy discloses small, poorly differentiated cells with scant cytoplasm, hyperchromic nuclei, and inconspicuous nucleoli with surrounding red blood cells (hematoxylin and eosin, X300). (Right) Histopathologic examination of the mediastinal mass biopsy demonstrates a uniform proliferation of small, round to oval cells with a high nuclear to cytoplasmic ratio. The chromatin is stippled with small nucleoli. In addition, several mitotic figures are present (hematoxylin and eosin, X400).
performed to secure a diagnosis. The cytopathology disclosed a uniform population of cells with scanty cytoplasm with small, hyperchromic, round nuclei (Figure 2, left). Immunohistochemistry was positive for cytokeratin and chromogranin and negative for leukocyte common antigen, which was consistent with a diagnosis of small cell thymic carcinoma. The morphologic characteristics of the cells obtained on fine-needle aspiration biopsy were identical to those obtained from the mediastinal incisional biopsy that was performed in 1993, confirming the clinical sus picion of thymic carcinoma metastatic to the orbit (Figure 2, left). Palliative treatment with external beam radiation to the left orbit resulted in complete resolution of the patient's ocular signs and symptoms. However, the patient continued to feel unwell, with weakness and shortness of breath. Subsequently, he returned to his homeland in Singapore to seek alternative therapies and has been lost to follow-up. Thymic tumors are the most common neoplasms in the anterior mediastinum of adults.4 Derived from thymic epithelial cells, the majority of tumors are well circumscribed and encapsulated and do not 402
invade the surrounding structures. Thymic tumors are divided into two categories: thymomas and thymic carcinomas. Thymomas are classified as benign or malignant based not on cellular features but rather on evidence of macroscopic invasion at the time of surgery. Thymomas rarely metastasize, but when this does occur, the most frequent sites of involvement are bone, lymph nodes, and liver. Rarely, thymic epitheli al neoplasms display obvious cytologic malignant features and are then termed "thymic carcinoma."5 The mortality rate of thymic carcinoma is much higher than that of thymoma, and a clear separation of these two entities is therefore justified.5 This case demonstrates the usefulness of fine-needle aspiration biopsy in confirming a suspected clinical diagnosis with minimal patient discomfort and morbidity. Rare tumors can be confidently diagnosed with fine-needle aspiration biopsy with minimal tissue, particularly in cases in which the biopsy of the primary tumor has already been obtained. REFERENCES 1. Shields CL, Shields JA, Peggs M. Tumors metastatic to the orbit. Ophthalmic Plast Reconstr Surg 1988;4:73-80.
AMERICAN JOURNAL OF OPHTHALMOLOGY
SEPTEMBER 1997
2. Freedman MI, Folk JC. Metastatic tumors to the eye and orbit: patient survival and clinical characteristics. Arch Ophthalmol 1987;105:1215-1219. 3. Font RL, Ferry AP. Carcinoma metastatic to the eye and orbit, III: a clinicopathological study of 28 cases metastatic to the orbit. Cancer 1976;38:1326-1335. 4. Tomiak EM, Evans WK. The role of chemotherapy in invasive thymoma: a review of the literature and consideration for future clinical trials. Crit Rev Oncol Hematol 1993; 15: 113-124. 5. Troung LD, Mody DR, Cagle PT, Jackson-York GL, Schwartz MR, Wheeler TM. Thymic carcinoma: a clinicopathologic study of 13 cases. Am J Surg Pathol 1990;14:151-166.
Acquired Capillary Hemangioma of the Eyelid in an Adult Brent A. Murphy, MD, Gamil S. Dawood, MD, and Curtis E. Margo, MD
FIGURE 1. A 7-mm blue nodule (arrows) is present in the outer portion of the right lower eyelid. The lesion had a rubbery consistency.
PURPOSE: To report a case of an acquired capil lary hemangioma of the eyelid in an adult. METHODS: A 38-year-old man developed a dis crete blue nodular mass in the lower eyelid. The lesion grew slowly for 6 months and then remained stable in size. Excisional biopsy was performed. RESULT: Histopathologic examination of an exci sional biopsy specimen of the nodular mass dis closed a capillary hemangioma. CONCLUSIONS: Capillary hemangioma of the eyelid can occur in adults but should be distin guished from other acquired vascular lesions, in cluding angiosarcoma.
C
APILLARY HEMANGIOMA IS THE MOST COMMON congenital vascular tumor of the periorbital re gion.1 Most capillary hemangiomas appear at birth or . within the first weeks of life. They often display exuberant growth over the next 6 to 12 months and may result in occlusion of the visual axis or anisometropic amblyopia. This growth phase is followed by spontaneous regression during the first decade of life. Capillary hemangioma has not been recognized as an acquired periorbital lesion in adults.
Accepted for publication April 28, 1997. Departments of Ophthalmology (B.A.M.) and Medicine (G.S.D.), University of South Alabama, and Department of Ophthalmology (C.E.M.), University of South Florida. Inquiries to Brent A. Murphy, MD, P.O. Box 8448, Mobile, AL J6689-0448. VOL.124, No. 3
FIGURE 2. Capillary hemangioma showing closely packed vascular spaces lined by uniform endothelial cells and scant extracellular matrix (hematoxylin and eosin, X390). (Inset) Low magnification of biopsy specimen showing the cellularity and well-circumscribed border of the vascular tumor (hematoxylin and eosin, X16).
A 38-year-old m a n was referred for evaluation of a
lower eyelid tumor. The lesion was first noted 1 year earlier as a small dark spot in the eyelid. The lesion had enlarged for 6 months and then remained stable in size. Increased vascularity of the overlying skin and blue discoloration had developed during the ensuing months. There were no associated ophthalmic symp toms, other cutaneous lesions, or history of anteced ent trauma. External examination showed a blue subcutaneous nodule in the right lower eyelid with
BRIEF REPORTS
403