Mycosis Fungoides Jamie L. Zucker, MD, Michael F.
Doyle, MD
\s=b\ We report a case of mycosis fungoides metastatic to the anterior orbit in an 83-year-old woman. The patient had a history of several years' duration of well\x=req-\ controlled primary mycosis fungoides with localized dermatologic T-cell lymphomas, mainly on the lower extremities. She developed gradual proptosis, hyperophthalmos, and vertical diplopia. Computed tomography demonstrated a solid, extraconal mass in the right lower anterior orbit. Biopsy revealed a gristly, yellowish, infiltrating mass at the level of the inferior orbital septum. Pathologic examination revealed anaplastic T lymphocytes with multiloculated, cerebri-
form nuclei that matched those of the primary mycosis fungoides skin tumors. Mycosis fungoides rarely spreads to the ocular and periocular structures, and this case was unique in that it presented as a pure orbital lesion with sparing of the overlying lids and adnexal structures.
(Arch Ophthalmol. 1991;109:688-691)
TX/Tycosis fungoides
is
a
Metastatic to the Orbit
cutaneous
T-cell
lymphoid tumor that may spread to involve multiple extracutaneous tissues. The lymph nodes and viscera, especially the lung, spleen, and liver, are most commonly in¬
Central nervous system and ocular manifestations are rare and may only be found at autopsy.3"" Of the ocular findings, eyelid tumors are most commonly reported.3 Other
volved.1'2
findings include keratitis, uve¬ itis, papilledema and optic atrophy, conjunctival tumors, and vitreoretinal ocular
involvement.3'" Orbital involvement has been reported in several cases, either as a posterior extension from an
eyelid tumor or as an autopsy finding. Very few cases of pure orbital involve¬ ment without periocular skin involve¬ ment have been reported. We describe a patient who developed extracutaneous mycosis fungoides presenting as an
extraconal orbital
mass
REPORT OF A CASE An 83-year-old vornan had a history of mycosis fungoidea of several years' dura¬
tion. She had been followed for intermit¬
tent, recurring, erythematous plaques
for publication December 10, 1990. From the Departments of Surgery (Dr Zucker) and Pathology (Dr Doyle), Timken Mercy Medical Center, Canton, Ohio. Reprint requests to Canton Ophthalmology Associates Inc, 2912 W Tuscarawas, Canton, OH 44708 (Dr Zucker).
on
the trunk and lower extremity. Her com¬ plete blood cell counts had remained nor¬ mal, with no evidence of mycosis cells in the blood (Sézary syndrome). She had been treated with topical nitrogen mustard on multiple cutaneous areas, with good re¬ sults. Roentgenographie evaluation showed no evidence of visceral involvement. Com¬ puted tomography of the abdomen, chest, and pelvis done in September 1988 showed a possible goiter or an enlarged neck lymph node. A biopsy was not performed at the time. Because of recurring lower-extremity le¬ sions, chemotherapy was initiated in Au¬ gust 1989, consisting of chlorambucil (Leukeran; 2 mg/d). Due to a poor response, treatment was switched to vincristine sul¬ fate (Oncovin; 1 mg) administered intrave¬ nously once per month. In November 1989,
cyclophosphamide (Cytoxan;
Accepted
without
overlying eyelid or adnexal changes.
50
mg/d)
was
added to the regimen. The patient had several typical mycosis fungoides plaques on her right lower ex¬ tremity in the ankle area. These were erythematous, well-circumscribed plaques
measuring approximately 4 4 cm (Figs 1 and 2). One was slightly ulcerated in the
presentation
to one of us 1-month history of vertical diplopia and mild discomfort and tenderness of the right lower lid area. She also complained of mild tearing and a slight¬ ly red right eye. She denied previous or current eyelid lesions and believed that her visual acuity was not affected by this mass. She denied eye pain, trauma, metamor¬ phopsia, scotomas, discharge, or photopho¬ bia. The examination revealed visual acuity of 20/60 OD and 20/60 OS. A firm, slightly tender, 3.5x2.0-cm, deep, right, inferior oi'bital mass was felt (Figs 3 and 4) at about the level of the inferior orbital rim. The overlying eyelid was essentially normal with no evidence of cutaneous malignancy. Pupils were normally round and reactive, with no afferent defect. The bulbar conjunc¬ tiva was slightly injected with prominent vessels. An obvious right hypertropia mea¬ sured 10 prism diopters (D). The right eye was elevated about 5 mm above the level of the left eye. Motility of the right globe was very restricted. There was moderate limita¬ tion of abduction and essentially no down¬ ward action in any field (Fig 5). Hertel exophthalmometry measured 20 mm in the right eye and 17 mm in the left eye, with a base of 93 mm. The results of the slit-lamp examination were otherwise normal with 2 + nuclear sclerotic lens changes. The pos¬ terior segment examination results were also normal, with no vitreal opacities or chorioretinal folds and a 0.4 cup-disc ratio. A computed tomographic scan showed a dense, homogeneous, extraconal orbital mass interiorly on the right eye between the globe and the orbital floor (Figs 6 and 7). The globe itself appeared to be spared. However, it was mildly proptotic and elevated by the mass. The intraconal and intracranial structures were normal. The patient underwent an orbital biopsy through a right infraciliary lid incision. A biopsy of the leg lesion was also performed. The overlying tissues were normal, and a gristly, yellow, firm mass was found at the level of the inferior orbital septum. Frozen
center.
On
(J.L.Z.), the patient had
Downloaded From: http://archopht.jamanetwork.com/ by a University of California - San Diego User on 01/25/2016
a
and permanent sections were obtained; my¬ cosis fungoides metastatic to the orbit was found and radiation therapy to the orbit was begun, with early shrinkage of the tumor.
PATHOLOGIC FINDINGS Gross examination of the leg skin biopsy specimen revealed an elliptical portion of focally erythematous, scaly, gray-red skin measuring 120 mm horizontally, 20 mm vertically, and 10 mm anteroposteriorly. Microscopic examination of this specimen showed some hyperkeratosis and parakeratosis. The underlying stratified squamous epithelium was flattened and the underly¬ ing papillary and subpapillary dermis con¬
tained sheets and bands of dark small and medium-sized mononuclear cerebriform cells. Many of these cells had prominent nucleoli, and scattered mitoses were seen. There was extensive tumor involvement of the dermoepidermal junction along with involvement of the underlying skin appen¬ dages. Epidermotrophism defined by the presence within the epidermis of scattered, single, mononuclear cells surrounded by a halolike clear space was noted. In some areas, the characteristic Pautrier microabscesses were present (Fig 8), which con¬ sisted of small, intraepidermal groups of tightly aggregated mononuclear cells lo¬ cated within a vacuole. Gross examination of the connective tis¬ sue specimen from the lower right orbit revealed multiple, granular, dull, graywhite and gray-tan, spongy tissue frag¬ ments. The two larger fragments together measured 40 mm horizontally, 30 mm verti¬ cally, and 10 mm anteroposteriorly. No
skin, eyelashes, cysts, noted.
or
pigmentation
Fig 1. —Lower-extremity cutaneous mycosis fungoides plaque at the time of orbital spread.
Fig 2.—Another lower-extremity mycosis fun¬ goides lesion with slight central ulcération.
Fig 3.—Clinical photograph (front view) showing proptosis and inferior orbital mass on the right side. The lid and adnexal struc¬
Fig 4. —Clinical photograph (side view) show¬ ing right anterior orbital mass.
was
Microscopic examination of the right or¬ specimen showed fibrocollagenous and fibrofatty tissue that contained vessels, small nerve twigs, and rare fragments of bital
striated muscle. All of these structures were infiltrated by cerebriform mononucle¬ ar cells similar to those seen in the leg biopsy specimen described above (Fig 9). Cells in some areas had a characteristic perivascular arrangement. A touch imprint of the infiltrate was obtained that showed characteristic mycosis cells (Fig 10). Immunoperoxidase stains revealed that the cerebriform mononuclear cells were leuko¬ cyte common antigen and UCHL-1 positive, and therefore lymphocytes. These results are highly suggestive of mycosis fungoides, a T-cell lymphoma found in both the leg and orbital tissue specimens.
tures are normal and have no cutaneous tumors.
Fig 5. —Clinical photograph demonstrating marked restriction of downward gaze of the due to orbital tumor mass.
COMMENT
Mycosis fungoides is a malignant T-cell cutaneous lymphomatous dis¬ ease that occurs in middle-aged or elderly individuals. It typically follows an indolent, progressive course and responds variably to both topical and systemic chemotherapeutic agents.12 Clinically, the lesions are usually ery¬ thematous, eczematous, flat patches or plaques covering the torso or extrem-
Downloaded From: http://archopht.jamanetwork.com/ by a University of California - San Diego User on 01/25/2016
right eye
dense
Fig 7.—Coronal computed tomographic view of the orbital mass on the right side.
8.—View of right leg lesion showing Pautrier microabscess and involvement of the dermoepidermal junction (hematoxylin-eosin, origi¬ nal magnification 100).
Fig 9.—View of the right orbital biopsy specimen showing infiltration by convoluted large and small cells of mycosis fungoides (hematoxylin-
Fig 6.—Computed tomographic
extraconal anterior orbital
Fig
scan
(axial view) showing
mass.
eosin, original magnification
ities that may spread over more skin. Later stages demonstrate cutaneous tumors, ulcérations, and extracutaneous spread. A rare variant of mycosis fungoides is the Sézary syndrome with skin lesions along with hepatosplenomegaly and abnormal lymphocytes (Sézary cells) circulating in the blood. Extracutaneous dissemination of the tumor was once thought to be uncom¬ mon, but recent reports show visceral spread in up to 82% of autopsy find¬ ings.' Lymph nodes, viscera, and cen-
Fig 10.—Touch imprint from the orbital lesion showing the characteristic cerebriform lym¬ phocyte of mycosis fungoides (WrightGiemsa, original magnification 100).
100).
nervous system are involved most often; however, almost all organs and
trai
tissues
were
found to be involved with on careful autopsy
mycosis fungoides
study.12
Histopathologic features of mycosis fungoides involving both the skin and
sites are similar.1,2 Bandlike cellular infiltrates of variable density involve the superficial dermis and focally invade the epidermis. The infiltrate contains a mixture of inflam¬ matory cell types but must contain the atypical lymphoid cells of variable size known as Lutzner or mycosis cells. They have a densely hyperchromatic nucleus that has a crenated appear¬ ance. This is due to deeply clefted, multilobulated, cerebriform nuclei of extracutaneous
Downloaded From: http://archopht.jamanetwork.com/ by a University of California - San Diego User on 01/25/2016
these cells. Epidermal involvement by the infiltrate is a requisite for the diagnosis of mycosis fungoides. Indi¬ vidual cells may be seen in the epider¬ mis or clusters known as Darier-Pautrier abscesses. Extracutaneous sites, including the ocular and periocular tis¬ sue, also demonstrate these character¬ istically atypical lymphocytes and infiltrates, along with occasional Pau¬ trier microabscesses.1,2 Various ocular findings in mycosis fungoides have been reported. A re¬ view of the literature3 showed anterior segment involvement to be most com¬ mon, with eyelid tumors most fre¬ quently found. Other tissues, such as the conjunctiva, caruncle, cornea, sclera, uvea, retina, and optic nerve, have been reported to contain mycosis
fungoides.3'6"8
Orbital involvement of mycosis fun¬ has been reported as autopsy
goides
patients,3"11 with little premortem abnormality. Another re¬ findings
of four
port described
a patient with massive anterior orbital involvement that in¬ cluded the eyelid.12 Most reports of orbital involvement appear to involve the spread of eyelid lesions posteriorly to the anterior orbit, with very few of primarily orbital origin. One report13 described a patient with large subcuta¬ neous tumors of the connective tissues involving the orbit, flank, buttock, and neck. The authors described these as "nonepidermotropic" tumors of ad¬ vanced mycosis fungoides, a very atyp¬ ical course of the disease. Our patient is an elderly woman with a history of several years' dura¬ tion of cutaneous mycosis fungoides of the torso and lower extremity. She was free of symptomatic, hématologie,
roentgenographic extracutaneous spread. She presented with what or
to be purely orbital involve¬ of mycosis fungoides, with evidence of eyelid skin involve¬
appeared ment no
so-called nonepidermotropic Roentgenographic and micro¬ scopic studies confirmed the diagnosis of mycosis fungoides, with mycosis
ment,
a
tumor.
cells and infiltrates
seen
in the orbital
biopsy specimen. Certainly, this is a most unusual initial presentation for extracutaneous mycosis fungoides spread. The treat¬ ment and response to ocular spread of this tumor vary, but treatment usually consists of topical nitrogen mustard applied to the eyelids, local radiothera¬ py for deeper or intraocular lesions, and possibly systemic steroid-immunosuppressive therapy. Our patient re¬ sponded well to local radiotherapy to the orbit, with shrinkage of the tumor and improvement of ocular mass symptoms.
References 1. Long JC, Mihm MC. Mycosis fungoides with extracutaneous dissemination: a distinct clinico-
pathologic entity. Cancer. 1974;34:1745-1755. 2. Rappaport H, Thomas LB. Mycosis fungoides: the pathology of extracutaneous involvement. Cancer.
1974;34:1198-1229.
Ramsay DL. Ocular findings in mycosis fungoides. Arch Ophthalmol. 1981;99: 3. Stenson S,
272-277. 4. Hauch TW, Shelbourne JD, Cohen HJ, Mason D, Kremer WB. Meningeal mycosis fungoides: clinical and cellular characteristics. Ann Intern Med.
1975;82:499-505. 5. Weber MD, McGavran MH. Mycosis fungoides involving the brain. Arch Neurol. 1967;
16:645-650. 6. Pariser DM. Mycosis fungoides involving the brain and optic nerves. Arch Dermatol. 1978; 114:397-399. 7. Keltner JL, Fritsch E, Cykiert RC, Albert DM. Mycosis fungoides intraocular and central nervous system involvement. Arch Ophthalmol. 1977; 95:645-650. 8. Forster HC. Mycosis fungoides with intraocular involvement. Trans Am Acad Ophthalmol Oto-
laryngol. 1960;64:308-313. 9. Brewitt H, Hartung J, Hoffman K. Lid und orbitabelteiligung bei mycosis fungoides. Klin Monatsbl Augenheilkd. 1974;164:345-349. 10. Pau H, Tillmann W, Goerz G. Mycosis fun-
goides affecting the eye and internal organs. Klin Monatsbl Augenheilkd. 1976;168:706-712. 11. Giannoti B, Lampis R, Scialdone D. Micosi fungoide a primitiva localizzazione orbitaria. Ann Ottal. 1963;89:483-492. 12. Meekins B, Proia AD, Klintworth GK. Cutaneous T-cell lymphoma presenting as a rapidly enlarging ocular adnexal tumor. Ophthalmology. 1985;92:1288-1293. 13. Whitbeck EG, Spiers ASD, Hussain M. My-
cosis fungoides: subcutaneous and visceral tumors, orbital involvement, and ophthalmoplegia. J Clin Oncol. 1983;1:270-276.
Currently in Other AMA Journals ARCHIVES OF INTERNAL MEDICINE
Progression of Peripheral Occlusive Arterial Disease in Diabetes Mellitus: What Factors Are Predictive? P. J. Palumbo, MD; W. Michael O'Fallon, PhD; Philip J. Osmundson, MD; Bruce R. Zimmerman, MD; Alice L. Langworthy; Francis J. Kazmier, MD (Arch Intern Med. 1991;151:717-721) Bruceila Optic Neuritis Mohamed Abd Elrazak, MD (Arch Intern Med. 1991;151:775-778)
Downloaded From: http://archopht.jamanetwork.com/ by a University of California - San Diego User on 01/25/2016
Doyle, MD
\s=b\ We report a case of mycosis fungoides metastatic to the anterior orbit in an 83-year-old woman. The patient had a history of several years' duration of well\x=req-\ controlled primary mycosis fungoides with localized dermatologic T-cell lymphomas, mainly on the lower extremities. She developed gradual proptosis, hyperophthalmos, and vertical diplopia. Computed tomography demonstrated a solid, extraconal mass in the right lower anterior orbit. Biopsy revealed a gristly, yellowish, infiltrating mass at the level of the inferior orbital septum. Pathologic examination revealed anaplastic T lymphocytes with multiloculated, cerebri-
form nuclei that matched those of the primary mycosis fungoides skin tumors. Mycosis fungoides rarely spreads to the ocular and periocular structures, and this case was unique in that it presented as a pure orbital lesion with sparing of the overlying lids and adnexal structures.
(Arch Ophthalmol. 1991;109:688-691)
TX/Tycosis fungoides
is
a
Metastatic to the Orbit
cutaneous
T-cell
lymphoid tumor that may spread to involve multiple extracutaneous tissues. The lymph nodes and viscera, especially the lung, spleen, and liver, are most commonly in¬
Central nervous system and ocular manifestations are rare and may only be found at autopsy.3"" Of the ocular findings, eyelid tumors are most commonly reported.3 Other
volved.1'2
findings include keratitis, uve¬ itis, papilledema and optic atrophy, conjunctival tumors, and vitreoretinal ocular
involvement.3'" Orbital involvement has been reported in several cases, either as a posterior extension from an
eyelid tumor or as an autopsy finding. Very few cases of pure orbital involve¬ ment without periocular skin involve¬ ment have been reported. We describe a patient who developed extracutaneous mycosis fungoides presenting as an
extraconal orbital
mass
REPORT OF A CASE An 83-year-old vornan had a history of mycosis fungoidea of several years' dura¬
tion. She had been followed for intermit¬
tent, recurring, erythematous plaques
for publication December 10, 1990. From the Departments of Surgery (Dr Zucker) and Pathology (Dr Doyle), Timken Mercy Medical Center, Canton, Ohio. Reprint requests to Canton Ophthalmology Associates Inc, 2912 W Tuscarawas, Canton, OH 44708 (Dr Zucker).
on
the trunk and lower extremity. Her com¬ plete blood cell counts had remained nor¬ mal, with no evidence of mycosis cells in the blood (Sézary syndrome). She had been treated with topical nitrogen mustard on multiple cutaneous areas, with good re¬ sults. Roentgenographie evaluation showed no evidence of visceral involvement. Com¬ puted tomography of the abdomen, chest, and pelvis done in September 1988 showed a possible goiter or an enlarged neck lymph node. A biopsy was not performed at the time. Because of recurring lower-extremity le¬ sions, chemotherapy was initiated in Au¬ gust 1989, consisting of chlorambucil (Leukeran; 2 mg/d). Due to a poor response, treatment was switched to vincristine sul¬ fate (Oncovin; 1 mg) administered intrave¬ nously once per month. In November 1989,
cyclophosphamide (Cytoxan;
Accepted
without
overlying eyelid or adnexal changes.
50
mg/d)
was
added to the regimen. The patient had several typical mycosis fungoides plaques on her right lower ex¬ tremity in the ankle area. These were erythematous, well-circumscribed plaques
measuring approximately 4 4 cm (Figs 1 and 2). One was slightly ulcerated in the
presentation
to one of us 1-month history of vertical diplopia and mild discomfort and tenderness of the right lower lid area. She also complained of mild tearing and a slight¬ ly red right eye. She denied previous or current eyelid lesions and believed that her visual acuity was not affected by this mass. She denied eye pain, trauma, metamor¬ phopsia, scotomas, discharge, or photopho¬ bia. The examination revealed visual acuity of 20/60 OD and 20/60 OS. A firm, slightly tender, 3.5x2.0-cm, deep, right, inferior oi'bital mass was felt (Figs 3 and 4) at about the level of the inferior orbital rim. The overlying eyelid was essentially normal with no evidence of cutaneous malignancy. Pupils were normally round and reactive, with no afferent defect. The bulbar conjunc¬ tiva was slightly injected with prominent vessels. An obvious right hypertropia mea¬ sured 10 prism diopters (D). The right eye was elevated about 5 mm above the level of the left eye. Motility of the right globe was very restricted. There was moderate limita¬ tion of abduction and essentially no down¬ ward action in any field (Fig 5). Hertel exophthalmometry measured 20 mm in the right eye and 17 mm in the left eye, with a base of 93 mm. The results of the slit-lamp examination were otherwise normal with 2 + nuclear sclerotic lens changes. The pos¬ terior segment examination results were also normal, with no vitreal opacities or chorioretinal folds and a 0.4 cup-disc ratio. A computed tomographic scan showed a dense, homogeneous, extraconal orbital mass interiorly on the right eye between the globe and the orbital floor (Figs 6 and 7). The globe itself appeared to be spared. However, it was mildly proptotic and elevated by the mass. The intraconal and intracranial structures were normal. The patient underwent an orbital biopsy through a right infraciliary lid incision. A biopsy of the leg lesion was also performed. The overlying tissues were normal, and a gristly, yellow, firm mass was found at the level of the inferior orbital septum. Frozen
center.
On
(J.L.Z.), the patient had
Downloaded From: http://archopht.jamanetwork.com/ by a University of California - San Diego User on 01/25/2016
a
and permanent sections were obtained; my¬ cosis fungoides metastatic to the orbit was found and radiation therapy to the orbit was begun, with early shrinkage of the tumor.
PATHOLOGIC FINDINGS Gross examination of the leg skin biopsy specimen revealed an elliptical portion of focally erythematous, scaly, gray-red skin measuring 120 mm horizontally, 20 mm vertically, and 10 mm anteroposteriorly. Microscopic examination of this specimen showed some hyperkeratosis and parakeratosis. The underlying stratified squamous epithelium was flattened and the underly¬ ing papillary and subpapillary dermis con¬
tained sheets and bands of dark small and medium-sized mononuclear cerebriform cells. Many of these cells had prominent nucleoli, and scattered mitoses were seen. There was extensive tumor involvement of the dermoepidermal junction along with involvement of the underlying skin appen¬ dages. Epidermotrophism defined by the presence within the epidermis of scattered, single, mononuclear cells surrounded by a halolike clear space was noted. In some areas, the characteristic Pautrier microabscesses were present (Fig 8), which con¬ sisted of small, intraepidermal groups of tightly aggregated mononuclear cells lo¬ cated within a vacuole. Gross examination of the connective tis¬ sue specimen from the lower right orbit revealed multiple, granular, dull, graywhite and gray-tan, spongy tissue frag¬ ments. The two larger fragments together measured 40 mm horizontally, 30 mm verti¬ cally, and 10 mm anteroposteriorly. No
skin, eyelashes, cysts, noted.
or
pigmentation
Fig 1. —Lower-extremity cutaneous mycosis fungoides plaque at the time of orbital spread.
Fig 2.—Another lower-extremity mycosis fun¬ goides lesion with slight central ulcération.
Fig 3.—Clinical photograph (front view) showing proptosis and inferior orbital mass on the right side. The lid and adnexal struc¬
Fig 4. —Clinical photograph (side view) show¬ ing right anterior orbital mass.
was
Microscopic examination of the right or¬ specimen showed fibrocollagenous and fibrofatty tissue that contained vessels, small nerve twigs, and rare fragments of bital
striated muscle. All of these structures were infiltrated by cerebriform mononucle¬ ar cells similar to those seen in the leg biopsy specimen described above (Fig 9). Cells in some areas had a characteristic perivascular arrangement. A touch imprint of the infiltrate was obtained that showed characteristic mycosis cells (Fig 10). Immunoperoxidase stains revealed that the cerebriform mononuclear cells were leuko¬ cyte common antigen and UCHL-1 positive, and therefore lymphocytes. These results are highly suggestive of mycosis fungoides, a T-cell lymphoma found in both the leg and orbital tissue specimens.
tures are normal and have no cutaneous tumors.
Fig 5. —Clinical photograph demonstrating marked restriction of downward gaze of the due to orbital tumor mass.
COMMENT
Mycosis fungoides is a malignant T-cell cutaneous lymphomatous dis¬ ease that occurs in middle-aged or elderly individuals. It typically follows an indolent, progressive course and responds variably to both topical and systemic chemotherapeutic agents.12 Clinically, the lesions are usually ery¬ thematous, eczematous, flat patches or plaques covering the torso or extrem-
Downloaded From: http://archopht.jamanetwork.com/ by a University of California - San Diego User on 01/25/2016
right eye
dense
Fig 7.—Coronal computed tomographic view of the orbital mass on the right side.
8.—View of right leg lesion showing Pautrier microabscess and involvement of the dermoepidermal junction (hematoxylin-eosin, origi¬ nal magnification 100).
Fig 9.—View of the right orbital biopsy specimen showing infiltration by convoluted large and small cells of mycosis fungoides (hematoxylin-
Fig 6.—Computed tomographic
extraconal anterior orbital
Fig
scan
(axial view) showing
mass.
eosin, original magnification
ities that may spread over more skin. Later stages demonstrate cutaneous tumors, ulcérations, and extracutaneous spread. A rare variant of mycosis fungoides is the Sézary syndrome with skin lesions along with hepatosplenomegaly and abnormal lymphocytes (Sézary cells) circulating in the blood. Extracutaneous dissemination of the tumor was once thought to be uncom¬ mon, but recent reports show visceral spread in up to 82% of autopsy find¬ ings.' Lymph nodes, viscera, and cen-
Fig 10.—Touch imprint from the orbital lesion showing the characteristic cerebriform lym¬ phocyte of mycosis fungoides (WrightGiemsa, original magnification 100).
100).
nervous system are involved most often; however, almost all organs and
trai
tissues
were
found to be involved with on careful autopsy
mycosis fungoides
study.12
Histopathologic features of mycosis fungoides involving both the skin and
sites are similar.1,2 Bandlike cellular infiltrates of variable density involve the superficial dermis and focally invade the epidermis. The infiltrate contains a mixture of inflam¬ matory cell types but must contain the atypical lymphoid cells of variable size known as Lutzner or mycosis cells. They have a densely hyperchromatic nucleus that has a crenated appear¬ ance. This is due to deeply clefted, multilobulated, cerebriform nuclei of extracutaneous
Downloaded From: http://archopht.jamanetwork.com/ by a University of California - San Diego User on 01/25/2016
these cells. Epidermal involvement by the infiltrate is a requisite for the diagnosis of mycosis fungoides. Indi¬ vidual cells may be seen in the epider¬ mis or clusters known as Darier-Pautrier abscesses. Extracutaneous sites, including the ocular and periocular tis¬ sue, also demonstrate these character¬ istically atypical lymphocytes and infiltrates, along with occasional Pau¬ trier microabscesses.1,2 Various ocular findings in mycosis fungoides have been reported. A re¬ view of the literature3 showed anterior segment involvement to be most com¬ mon, with eyelid tumors most fre¬ quently found. Other tissues, such as the conjunctiva, caruncle, cornea, sclera, uvea, retina, and optic nerve, have been reported to contain mycosis
fungoides.3'6"8
Orbital involvement of mycosis fun¬ has been reported as autopsy
goides
patients,3"11 with little premortem abnormality. Another re¬ findings
of four
port described
a patient with massive anterior orbital involvement that in¬ cluded the eyelid.12 Most reports of orbital involvement appear to involve the spread of eyelid lesions posteriorly to the anterior orbit, with very few of primarily orbital origin. One report13 described a patient with large subcuta¬ neous tumors of the connective tissues involving the orbit, flank, buttock, and neck. The authors described these as "nonepidermotropic" tumors of ad¬ vanced mycosis fungoides, a very atyp¬ ical course of the disease. Our patient is an elderly woman with a history of several years' dura¬ tion of cutaneous mycosis fungoides of the torso and lower extremity. She was free of symptomatic, hématologie,
roentgenographic extracutaneous spread. She presented with what or
to be purely orbital involve¬ of mycosis fungoides, with evidence of eyelid skin involve¬
appeared ment no
so-called nonepidermotropic Roentgenographic and micro¬ scopic studies confirmed the diagnosis of mycosis fungoides, with mycosis
ment,
a
tumor.
cells and infiltrates
seen
in the orbital
biopsy specimen. Certainly, this is a most unusual initial presentation for extracutaneous mycosis fungoides spread. The treat¬ ment and response to ocular spread of this tumor vary, but treatment usually consists of topical nitrogen mustard applied to the eyelids, local radiothera¬ py for deeper or intraocular lesions, and possibly systemic steroid-immunosuppressive therapy. Our patient re¬ sponded well to local radiotherapy to the orbit, with shrinkage of the tumor and improvement of ocular mass symptoms.
References 1. Long JC, Mihm MC. Mycosis fungoides with extracutaneous dissemination: a distinct clinico-
pathologic entity. Cancer. 1974;34:1745-1755. 2. Rappaport H, Thomas LB. Mycosis fungoides: the pathology of extracutaneous involvement. Cancer.
1974;34:1198-1229.
Ramsay DL. Ocular findings in mycosis fungoides. Arch Ophthalmol. 1981;99: 3. Stenson S,
272-277. 4. Hauch TW, Shelbourne JD, Cohen HJ, Mason D, Kremer WB. Meningeal mycosis fungoides: clinical and cellular characteristics. Ann Intern Med.
1975;82:499-505. 5. Weber MD, McGavran MH. Mycosis fungoides involving the brain. Arch Neurol. 1967;
16:645-650. 6. Pariser DM. Mycosis fungoides involving the brain and optic nerves. Arch Dermatol. 1978; 114:397-399. 7. Keltner JL, Fritsch E, Cykiert RC, Albert DM. Mycosis fungoides intraocular and central nervous system involvement. Arch Ophthalmol. 1977; 95:645-650. 8. Forster HC. Mycosis fungoides with intraocular involvement. Trans Am Acad Ophthalmol Oto-
laryngol. 1960;64:308-313. 9. Brewitt H, Hartung J, Hoffman K. Lid und orbitabelteiligung bei mycosis fungoides. Klin Monatsbl Augenheilkd. 1974;164:345-349. 10. Pau H, Tillmann W, Goerz G. Mycosis fun-
goides affecting the eye and internal organs. Klin Monatsbl Augenheilkd. 1976;168:706-712. 11. Giannoti B, Lampis R, Scialdone D. Micosi fungoide a primitiva localizzazione orbitaria. Ann Ottal. 1963;89:483-492. 12. Meekins B, Proia AD, Klintworth GK. Cutaneous T-cell lymphoma presenting as a rapidly enlarging ocular adnexal tumor. Ophthalmology. 1985;92:1288-1293. 13. Whitbeck EG, Spiers ASD, Hussain M. My-
cosis fungoides: subcutaneous and visceral tumors, orbital involvement, and ophthalmoplegia. J Clin Oncol. 1983;1:270-276.
Currently in Other AMA Journals ARCHIVES OF INTERNAL MEDICINE
Progression of Peripheral Occlusive Arterial Disease in Diabetes Mellitus: What Factors Are Predictive? P. J. Palumbo, MD; W. Michael O'Fallon, PhD; Philip J. Osmundson, MD; Bruce R. Zimmerman, MD; Alice L. Langworthy; Francis J. Kazmier, MD (Arch Intern Med. 1991;151:717-721) Bruceila Optic Neuritis Mohamed Abd Elrazak, MD (Arch Intern Med. 1991;151:775-778)
Downloaded From: http://archopht.jamanetwork.com/ by a University of California - San Diego User on 01/25/2016
