Histiocytoses

The histiocytoses include both Langerhans cell (Figure 15.7) and non-Langerhans cell proliferations.29'30 Langerhans cell histiocytosis (formerly known as histiocytosis X), is caused by a proliferation of Langerhans cells.31-33 Although both Langerhans cell histiocytosis and xanthogranuloma (XG) are characterized by the proliferation of histiocytes' significant morphologic and clinical differences exist because their cells of origin are class I and class II histiocytes, respectively.31

The light-microscopic features of Langerhans and non-Langerhans cell histiocytoses are quite similar, with a characteristic picture of histiocytic infiltration with lymphocytes, plasma cells, and multinucleated giant cells.32'33 Histopathologic variability is determinative of the age of the disease; the tumor ultimately scleroses toward the end of the process of pathology. Thus, it has been suggested that histiocytoses are more sensitive to treatment in the early stages.34

Both groups of histiocytoses show the same kind of pathology (Figure 15. 8). The landmark of the non-Langerhans type of proliferation was thought to be Touton-type multinucleated giant cells, but the absence of these cells does not rule out the diagnosis. Touton-type multinucleated giant cells may be seen in Langerhans cell histiocytosis as well, and, therefore, their presence or absence is not pathognomonic for either entity. The only certain way to specify the type of histiocytosis is to classify the histiocyte by electron microscopy and/or immunohistochemistry. Histiocytic lesions resulting from the proliferation of Langerhans cells are identified with ultrastructural depiction of intracytoplasmic Birbeck granules and pos-

TABLE 15.1. Correlation of MRI Enhancement Patterns with Histology.

Enhancement pattern

Tissue type

Homogeneous signal Tl, T2

and with Gd DPTA Homogeneous T1 signal changing to heterogeneous T2 signal High T2 signal Low T2 signal Intermediate T2 signal

Benign Malignant

Hypercellularity, calcification Hypocellularity, more collagen Difficult differential diagnosis

l£0 *«j 1 0 t*c traction suture covered with silicone tubing l£0 *«j 1 0 t*c

FIGURE 15.6. (A) Intraoperative and (B) CT appearance of a giant cell fibroblastoma. Note the well-delineated but not encapsulated fibrous tumor (T), which offers a very homogeneous appearance on CT because of its hypercellularity. The traction suture through the lateral rectus muscle was pulled medially for easy dissection of the lateral orbital mass; the silk suture was covered with silicone tubing to protect the cornea (K) during traction.

itive reaction to S-100 protein and CD1a. Non-Langer-hans cells express other surface markers, including blood clotting transglutaminase factor XIIIa, CD68, Mac387, and vimentin without Birbeck granules.30 These cell markers have been observed in juvenile xanthogranuloma (JXG), xanthoma disseminatum, and progressive nodular histocytosis.

Patients with Langerhans cell disease are generally younger than those with XG; the majority of these patients present between the ages of 1 and 15 years, with

FIGURE 15.7. Langerhans hystiocytosis (eosinophilic granuloma in a young child pushing the right eye inferiorly). (A) Irregular surface of the upper eyelid and superior periorbital skin is due to radiation treatment from which the child benefitted little. (B) Electron microscopic depiction of Langerhans histiocytes with Birbeck granules (white arrow). Identification of these cytoplasmic organelles confirms the tumor cells to be Langerhans histiocytes.

FIGURE 15.7. Langerhans hystiocytosis (eosinophilic granuloma in a young child pushing the right eye inferiorly). (A) Irregular surface of the upper eyelid and superior periorbital skin is due to radiation treatment from which the child benefitted little. (B) Electron microscopic depiction of Langerhans histiocytes with Birbeck granules (white arrow). Identification of these cytoplasmic organelles confirms the tumor cells to be Langerhans histiocytes.

FIGURE 15.8. (A) Intraoperative appearance of yellowish-orange xanthogranulomatous tumor through lateral orbitotomy. (B) Gross specimen from the same patient showing focally hemorrhagic, yellowish, fatty-looking tissue. Inset: Histopathologic appearance of the xanthogranuloma with diffuse infiltration of histiocytic cells and numerous Touton giant cells (T).

the peak ages being 2 to 4 years.32'35 The three classic forms are eosinophilic granuloma, which primarily affects bone; Hand-Schuller-Christian disease (a triad of exophthalmos, diabetes insipidus, and multiple bone lesions); and Letterer-Siwe disease (a syndrome of multiple soft tissue and bone involvement).36 It is recommended that a radiographic skeletal survey be obtained when Langerhans-cell histiocytosis is a diagnostic possibility. The lytic bone lesions have a predilection for the flat bones, most often involving the skull.37 However, radiologic findings in these diseases can be quite varied and include epiphyseal and transepiphyseal lesions, pathologic fractures, and dural extension of vertebral lesions. Less common findings include the involvement of the clavicle and small bones of the hands and feet and, rarely, ex-tracranial "button" sequestra, soft tissue calcification, and neurologic manifestations secondary to spinal dis-ease.38 When systemic (bone marrow, lung, liver, spleen, central nervous system, etc.) disease occurs, the prognosis of non-Langerhans histiocytosis is much worse.

Although histiocytosis X generally affects young children, rare cases have been observed in adults.39 These diseases usually present with destructive lesions in orbital and cranial bones associated with secondary soft tissue masses in the orbit.

Orbital involvement is most commonly seen with unifocal Langerhans cell disease (eosinophilic granuloma). A common site for eosinophilic granuloma is the anterior superior orbit. According to Woo and Harris, this is because frontal bone retains active bone marrow, which contains Langerhans cell precursors throughout childhood and adolescence.39 The same authors propose that osteolysis in eosinophilic granuloma is due to the production of prostaglandin E2 and interleukin 1 by the abnormal Langerhans cells. Their management recommendations include intralesional corticosteroid injection, which can inhibit the cyto-kines. The solitary lesions are best managed by de-bulking of the soft tissue mass with light debridement following an incisional biopsy to confirm the diagnosis. For multifocal, systemic involvement or in recurrent cases, systemic corticosteroids and/or antimetabolites or low-dose external beam radiation therapy (EBRT) are used.40

Orbital XG presents as a space-occupying lesion in the orbital and periorbital soft tissues and usually does not affect the orbital bones (Figures 15.9 and 15.10).41,42 The ocular signs and symptoms depend on the location and the size of the lesion. Generally, orbital XG presents without pain, but pain related to peripheral nerve origin may be the presenting symptom. The clinical differential diagnosis includes space-occupying lesions such as lymphoma, Sjogren's disease, and sarcoidosis, as well as other benign and malignant tumors depending on the age of the patient, laterality, and the location of the lesion (Figure 15.11). In most cases, orbital XG originates from the soft tissues; however, intracerebral XG affecting the orbit secondarily has been reported in both children and adults.43 The number of MR studies on orbital XG is limited, but this imaging technique usually reveals a destructive lesion with irregular, infiltrating margins of low signal intensity with scattered dark foci (Figure 15.12).43,44

CT studies reveal infiltrating, soft tissue masses within the orbit without bone destruction. Enlargement of extraocular muscles away from the lesion site and optic nerve entrapment within lesions have been described with CT examination.42 Some patients reveal Marcus-Gunn pupils, and others present visual field defects secondary to compressive optic neuropa-thy.45 The lacrimal gland may be involved as an extension of infiltrating masses. In our experience, one patient who presented with bilateral lacrimal gland enlargement was falsely diagnosed as having Sjogren's disease because of primary lacrimal gland pathology without other orbital lesions.

Ultrasonography shows irregular soft tissue masses with low-density areas most likely corresponding to necrotic foci, but this is not particularly useful for differential diagnosis. Orbital XG cannot be diagnosed from imaging findings. Rather, diagnosis should be based on histiopathologic, immunohistochemical, and electron microscopic studies. The usefulness of the CT, however, is significant in differential diagnosis of the lesion by allowing one to rule out bone damage and to delineate the extent of the lesion in order to plan surgical and radiation treatments. Furthermore, in some cases, the treatment response may be monitored by imaging studies.42

Erdheim-Chester disease (E-Cd) must be ruled out in every case of XG in the eyelids and orbit.46-48 While the pathogenesis of E-Cd remains unknown, it has recently been reported that the histiocytic element of XG is monoclonal, which suggests that these lesions may be neoplastic. This disease may therefore be con-

FIGURE 15.9. Bilateral proptosis and xanthalesmas of upper eyelids and medial canthi in a patient with Erdheim-Chester disease.

FIGURE 15.10. (A) Axial CT scan showing the homogeneous appearance of xanthogranuloma diffusely involving both orbits. The patient's vision was light perception in both eyes on admission. (B) X-ray of the humerus of the same patient with Erdheim-Chester disease revealing typical lytic and sclerotic changes. (C) Radionu-cleotide scan of the same patient with increased uptake in orbits.

lungs, heart (conduction network), kidneys, spleen, lymph nodes, and nervous system.46,50,51 In contrast to the asymmetric, lytic flat bone lesions of Langerhans histiocytosis, patients with E-Cd typically have polyostotic lytic and sclerotic lesions of long bones, with sparing of the appendicular skeleton. X-rays to detect metaphyseal sclerolytic destruction of the long bones, typical for E-Cd, can be easily and inexpensively employed as a screening measure. Systemic involvement should be considered in all cases of orbital XGs, and long bone films should be obtained.

Granulomatous histiocytic infiltrates have been described in the retroperitoneal tissue of E-Cd patients, causing serious renal complications ranging from hydronephrosis to renal failure.52,53 Although retroperitoneal XG has rarely been reported to regress spontaneously, in many instances it leads to obstructive uropathy, renal failure, and eventual death. The most common treatment measures are surgical excision combined with EBRT and oral steroids, but the prognosis in both local and systemic disease is usually not good.

FIGURE 15.10. (A) Axial CT scan showing the homogeneous appearance of xanthogranuloma diffusely involving both orbits. The patient's vision was light perception in both eyes on admission. (B) X-ray of the humerus of the same patient with Erdheim-Chester disease revealing typical lytic and sclerotic changes. (C) Radionu-cleotide scan of the same patient with increased uptake in orbits.

sidered to be the monocytic counterpart to the dendritic monoclonal expansion of non-Langerhans histio-cytosis. The same report suggests that this clonal expansion may also be secondary to an infectious agent, similar to the induction of gastric lymphoma by Helicobacter pylori.47

E-Cd was first described by Chester in 1930 as an unusual "lipidosis" with distinctive bone changes.49 Although rarely seen, E-Cd represents the most common form of systemic lipogranulomatosis, predominantly affecting adults, whereas Langerhans histiocy-tosis is a disease of childhood. E-Cd presents with histiocytic infiltrates involving retroperitoneal soft tissues, long bones, and other viscera including the

FIGURE 15.11. (A,B) Bilateral xanthogranulomas in superior lateral orbit. The patient later developed multiple skin nodules of the face but no systemic disease. Because of the bilateral superior lateral masses, she was considered to have Sjogren's disease at initial presentation. Later the tissue diagnosis obtained from these lesions revealed the true nature of the pathology. Note the glistening, yellow-orange cut surface of the well-delineated mass from the right superior orbit.

FIGURE 15.11. (A,B) Bilateral xanthogranulomas in superior lateral orbit. The patient later developed multiple skin nodules of the face but no systemic disease. Because of the bilateral superior lateral masses, she was considered to have Sjogren's disease at initial presentation. Later the tissue diagnosis obtained from these lesions revealed the true nature of the pathology. Note the glistening, yellow-orange cut surface of the well-delineated mass from the right superior orbit.

FIGURE 15.12. Axial and coronal MRIs with T1- and T2-weighted images, respectively, showing a mixture of hypo- and hyperintense signals in the superior medial aspect of the globe. One can deduce only that the orbit harbors an infiltrating mass lesion; the appearance is not diagnostic for any specific tumor. Histopathology proved the tumor to be a xanthogranuloma.

FIGURE 15.12. Axial and coronal MRIs with T1- and T2-weighted images, respectively, showing a mixture of hypo- and hyperintense signals in the superior medial aspect of the globe. One can deduce only that the orbit harbors an infiltrating mass lesion; the appearance is not diagnostic for any specific tumor. Histopathology proved the tumor to be a xanthogranuloma.

Ophthalmologists are most familiar with JXG, a self-limiting disorder of the skin and the iris that may be present at birth, but typically arises during infancy.54 The lesions consist of several yellow-orange cutaneous nodules on the skin of the head and neck, with the most frequent site of extracutaneous involvement being the uvea. This manifests as a yellowish iris mass, which can cause hyphema and glaucoma. Histopathologically, these are xanthogranulomatous lesions characterized by histiocytic proliferations associated with lymphocytes and Touton giant cells.32,54 JXG also develops in the orbit as isolated lesions.55 Whether these cases represent solitary JXG lesions without other manifestations of the disease or are orbital XGs occurring in children is open to speculation.

Another type of xanthogranulomatous disease involving the orbit is necrobiotic xanthogranuloma (Figure 15.13).56 This rare histiocytic disorder presents with indurated, yellowish-red, nontender skin nodules in the eyelids and periorbital structures. It is a progressive and destructive disease often associated with multiple organ involvement, paraproteinemia, and hematologic and/or lymphoproliferative malignant disorders. The granulomas are made of histio-cytes, lymphocytes, and Touton and foreign body giant cells intermixed with focal areas of necrosis. The ocular manifestations are varied and may include eyelid nodules, episcleritis, uveitis, iritis, keratitis, cellulitis, and proptosis.56 The differential diagnosis of necrobiotic XG includes JXG, granuloma annulare, foreign body granuloma, subcutaneous rheumatoid nodules, xanthoma disseminatum, primary and secondary amyloidosis, and E-Cd.56 These lesions tend to recur, or there may be an increase in inflammatory activity after incisional biopsy or surgical debulk-ing.57 Treatment involves chemotherapy with or without radiation and the avoidance of surgery where possible. Lifelong surveillance to detect associated malignancies is advised.

Cutaneous xanthomas may be encountered in patients with solitary orbital XG and in patients with E-Cd. These patients may or may not have elevated serum triglyceride and cholesterol levels. The most common form of cutaneous xanthoma is the xanthe-lasma palpebrum, also known simply as xanthelasma, which refers specifically to lesions that occur in the eyelids that may present as solitary or diffuse lesions (Table 15.2).58 Xanthelasmas consist of yellowish pla-coid skin lesions on the eyelids, usually bilateral, near the inner canthi. The prevalence rate is approximately 2 and 1% in women and men, respectively, increasing with age. Hyperlipidemia, usually Fredrickson hy-perlipidemic phenotype IIa, is present in approximately 50% of the patients with xanthelasmas. The histopathology of xanthelasma reveals a great deal of similarity to orbital XG, with aggregates of foamy his-tiocytes proliferating around the small blood vessels of the superficial dermis and a surrounding lympho-cytic reaction with occasional Touton giant cells. The pathogenesis of these lesions is unclear. It is thought that the cholesterol accumulation is derived from the blood, with the low-density lipids leaking through the capillary walls.59 However, several other factors are likely involved, inasmuch as more than half of hy-

FIGURE 15.13. Yellowish-red skin nodules of the eyelids and periorbital skin in necrobiotic xanthogranuloma.

FIGURE 15.13. Yellowish-red skin nodules of the eyelids and periorbital skin in necrobiotic xanthogranuloma.

TABLE 15.2. Clinical and Morphologic Features of Some Xanthomas.

Ophthalmic

Systemic

Xanthoma

Cell of origin

Histopathology

manifestations

manifestations

Xanthogranuloma

Non-Langerhans cell

Foamy histiocytes,

Solitary or multiple;

Seen in adults; long

Touton GCs,

eyelids, orbit,

bones, heart, kidney,

lymphocytes;

conjunctiva, optic

retroperitoneal lesions

cholesterol clefts

nerve

Juvenile

Non-Langerhans

Foamy histiocytes,

Solitary or multiple iris

Seen in infants;

xanthogranuloma

cell

lymphocytes, Touton

mass with hyphema,

skin and scalp

(JXG)

multinucleated GCs

cataract, glaucoma

lesions

Necrobiotic

Non-Langerhans cell?

Foamy histiocytes

Periorbital skin lesions,

Multiple violaceous

xanthogranuloma

with Touton and

eyelid and orbit mass,

skin nodules on

multinucleated GCs;

keratitis, episcleritis

face and trunk

necrosis

cholesterol

clefts

Planar xanthoma

Tissue macrophage

Foamy histiocytes,

Placoid skin lesions on

50% Hyperlipidemia

(xanthelasma)

(non-Langerhans cell?) Touton GCs,

eyelids

and lymphocyte

lymphocytes

Diffuse planar

Tissue macrophage

Foamy histiocytes,

Placoid skin lesions on

Confluent xanthelasmas

xanthoma

non-Langerhans cell?) Touton GCs,

eyelids

on trunk and face

and lymphocyte

lymphocytes

Progressive

Non-Langerhans cell

Spindly histiocytes,

Xanthomatous

Yellow, maculopapular

nodular

Touton GCs;

maculopapular lesions

skin lesions on face

histiocytosis

storiform pattern

on eyelids, ectropion

and trunk

Associated

Xanthoma

conditions

Workup

Laboratory

Treatment

Prognosis

Xanthogranuloma

Erdheim-Chester

Long bone films,

1 Cholesterol

Surgery,

Recurrent disease;

disease,

CT, MRI,

1 Triglyceride steroids,

very poor

periodontal

radionuclide

EBRT?

with E-Cd

problems

bone scan

Juvenile

Neurofi

Skin biopsy

Normal

Systemic

Spontaneous regression

xanthogranuloma

bromatosis?

and local

of systemic disease;

(JXG)

steroids;

poor visual

EBRT

prognosis with

hyphema 2°

glaucoma

Necrobiotic

Multiple

Immune

Leukopenia

Chemotherapy,

Poor with progressive

xanthogranuloma

myeloma,

profile

IgG gammopathy local

disease

lymphoma,

Cryoglobulinemia steroids,

PC dyscresias

minimal

surgery

Planar xanthoma

50% Hyper-

Serum lipid and

Serum lipid

No surgery,

50% recurrence after

(xanthelasma)

lipidemia,

cholesterol

chemotherapy

therapy

type II

levels

EBRT

Diffuse planar

40% Multiple

Serum lipid and

Serum lipid

Surgery,

50% recurrence after

xanthoma

myeloma,

cholesterol

CO2 laser,

therapy

leukemia,

levels

trichloracetic

lymphoma,

acid

cryoglobulinemia

Progressive

None

Skin biopsy

High uric acid Surgery

Spontaneous

nodular

regression

histiocytosis

perlipidemic patients do not develop these lesions, and normolipidemic patients sometimes do. Palpebral and periorbital xanthelasmas were present in three of our cases, including one with E-Cd. Serum cholesterol, lipid, and triglyceride levels were measured in six of our patients but were not helpful as diagnostic tests.

The most commonly used treatment modalities for XG lesions are surgical excision combined with oral corticosteroids and various chemotherapeutic agents, but recurrences are encountered often regardless of the size of the lesion.41,48,60-62 XG lesions are usually radiation resistant, but some patients are known to respond to EBRT (Figure 15.14). The prognosis in systemic involvement is very poor. According to one report, 22 of 59 E-Cd patients died within a mean of 32 months after diagnosis.31

FIGURE 15.14. A patient with bilateral xantho-granulomas before (A,B) and after (C) external beam radiation therapy. She did not have systemic disease and responded quite well to external beam radiation.

FIGURE 15.14. A patient with bilateral xantho-granulomas before (A,B) and after (C) external beam radiation therapy. She did not have systemic disease and responded quite well to external beam radiation.

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