Initial report of a cutaneous T-cell lymphoma appearing on the auricular helix.

Abstract

We report a case of cutaneous T-cell lymphoma in which the first sign of disease was involvement of the superior auricular helix. A review of the literature reveals that T-cell lymphoma often presents cutaneously, but it usually does not involve the ear, and an auricular lesion is rarely the first sign. The uncommon nature of this presentation, in addition to the potential need for multiple biopsies for a tissue diagnosis, can lead to a delay in diagnosis. When evaluating skin lesions in the head and neck, a high index of suspicion for cutaneous T-cell lymphoma is a key factor in its early diagnosis.

Introduction

Cutaneous T-cell lymphoma, previously known as mycosis fungoides, is primarily a malignancy of the T-helper (CD4+) cells. It usually occurs during the fourth to sixth decade of life. T-cell lymphoma often appears on the skin first, and it can progress to regional lymph nodes, the spleen, and almost any other organ. [1,2] T-cell lymphoma involves the skin early in the course of the disease. This early dermatologic involvement differentiates T-cell lymphoma from other malignant lymphomas, in which cutaneous lesions occur late and often signal an advanced stage. Within the head and neck, cutaneous T-cell lymphoma has been found in the hypopharynx, larynx, oropharynx, nasopharynx, oral cavity, maxillary sinus, mastoid, external and middle ear, and the cheek.

There are only a few reports in the literature of T-cell lymphoma that involved the ear. Ear involvement was first reported in 1971 by Strahan and Calcaterra. [3] They described a patient who had simultaneous involvement of the face, oropharynx, and external auditory canal. In 1975, Fenwick described a patient who had T-cell lymphoma involving the external auditory canal. [4]

Involvement of the auricular helix has not been previously described in the literature. Our case is unique in another respect, because it is the first reported case in which a patient had only auricular involvement.

Case report

An 87-year-old white man came to the emergency department at the Madigan Army Medical Center in Tacoma, Wash., complaining of left otalgia. The pain had been present for 1 month and had worsened over the previous week. There was no history of trauma, insect bites, or infection. The patient's medical history was negative for cancer, diabetes, and other potentially immunocompromising diseases. He denied having fever, chills, otorrhea, and vertigo.

The patient was seen in the otolaryngology clinic the following day. Examination of the left ear revealed an ulcerative lesion with purulent drainage on the superior helix. Laboratory values at that time showed a white blood cell count of 7.2 x [10.sup.9]/L with a normal differential, a hemoglobin of 14.0 g/dl, and a hematocrit of 42.5%. Liver enzymes and electrolytes were within normal limits. His chest x-ray was normal.

The patient was placed on oral antibiotics and the lesion was biopsied. The initial biopsy showed skin with ulceration and an inflammatory infiltrate within the superficial dermis. The inflammatory component was made up of a mixed population of lymphocytes and scattered eosinophils. Many features of chondrodermatitis nodularis chronica helicis were present, but no degenerative cartilage was seen. Immunohistochemical staining did not reveal kappa or lambda monoclonality within the B cells. T-cell markers showed a phenotypically normal T-cell population, suggesting a florid reactive process and not malignancy. The patient was continued on topical and oral antibiotics.

Approximately 5 weeks after his initial visit, the patient was admitted to the adult observation unit for unresolved left perichondritis. At that time, cultures demonstrated Staphylococcus aureus and Serratia marcescens. Consultation was obtained with an infectious disease specialist, who recommended appropriate intravenous antibiotics, which were initiated. The patient underwent a second biopsy, which showed a dense infiltrate of small to large transformed lymphocytes, some plasma cells, and very few eosinophils. Following 5 weeks of IV antibiotic therapy, the auricular lesion had not improved (figure 1). As a result, the patient was taken to the operating room for an excisional biopsy and primary reconstruction, with good results (figure 2). Following surgery, the results of immunohistochemistry for B- and T-cell markers became available. They revealed the presence of a predominant T-cell phenotype and some admixed B cells. The specimen was sent to the Armed Forces Institute of Pathology in Washington, DC, for T-cell receptor (TCR) gene rearrangement studies. These studies demonstrated dominant monoclonal rearrangements of the TCR [beta] and [gamma] genes.

The final pathology results of a third biopsy revealed a dense, diffuse infiltrate of atypical lymphocytes that extended from the mid-dermis through the underlying subcutaneous tissue and onto the adjacent cartilage (figure 3). The atypical lymphocytes were predominantly intermediate in size, although numerous small and large atypical cells were also present. The cells were characterized by moderate amounts of amphophilic cytoplasm, irregular and convoluted nuclei, and distinct nucleoli (figure 4). Immunohistochemical staining revealed that the neoplastic cells were T cells; they were CD45-, CD3-, and CD45RO-positive. TCR gene rearrangement studies were again performed on paraffin-embedded tissue, and dominant monoclonal rearrangements of the TCR [beta] and [gamma] genes were again detected. The morphologic, immunohistochemical, and gene rearrangement studies supported the diagnosis of cutaneous T-cell lymphoma.

Discussion

T-cell lymphoma has been reported to involve many areas of the head and neck, but an initial cutaneous presentation is less common. A review of the English-language literature reveals only three reports of cutaneous T-cell lymphoma involving the extemal auditory canal [3-5] and only two cases involving the cheek. [6,7] Our own review of the literature on cutaneous T-cell lymphoma involving the auricular helix failed to discover any similar case.

Classic cutaneous T-cell lymphoma generally, but not invariably, evolves through three defined clinical stages: the patch, plaque, and tumor stages. During the patch stage, lesions are no more than barely raised erythematous areas, often with adherent scales. These lesions are often confused with eczematous dermatitis. Early cutaneous T-cell lymphoma is often refractory to topical anti-inflammatory medications, and thus it usually causes clinical concern and demands a subsequent biopsy.

During the plaque stage, lesions are raised, indurated, and variable in color, ranging from pink to red to brown-purple. Well-defined plaques are common, and scaling and pruritus are often more prominent than they are in the patch stage. Patches and plaques of cutaneous T-cell lymphoma are relatively indolent, and they can progress over many years without ever spreading systemically.

If the disease goes untreated, tumor-stage nodules will eventually develop. These nodules are firm and dome-shaped, and they often affect the face, scalp, and body folds. The presence of tumor-stage nodules correlates with the visceral spread of the disease, and the prognosis is poor. Tumors infrequently arise de novo, a situation that is referred to as the "tumor d'emblee" form of cutaneous T-cell lymphoma.

The histologic diagnosis of cutaneous T-cell lymphoma in the patch stage is often one of the most difficult diagnoses that a pathologist is required to make. These lesions resemble a mild inflammatory dermatosis, and they feature a perivascular lymphocytic infiltrate in the papillary dermis and a mildly hyperplastic nonspongiotic epidermis that can exhibit focal hyperkeratosis or parakeratosis. Sparse numbers of atypical lymphocytes are scattered within the papillary dermis and epidermis. These lymphocytes can appear singly or in small aggregates (figure 3).

The plaque stage is characterized by a band-like infiltrate of polymorphous atypical lymphocytes within the papillary and superficial reticular dermis (figure 3). The larger transformed lymphocytes have hyperchromatic nuclei with irregular, convoluted nuclear membranes. Small aggregates of atypical lymphocytes are identified within the epidermis (Pautrier's microabscesses).

The tumor stage is often referred to as the non-epidermotropic stage because the papillary dermis and overlying epidermis are typically spared. Sheets and nodules of neoplastic lymphocytes are present within the reticular dermis. The lymphocytes range from small to large transformed cells, With admixed immunoblasts and even Reed-Sternberg-like cells. As noted earlier, the lymphocytes have hyperchromatic nuclei with the classic convoluted, cerebriform nuclear membranes. In the tumor stage, localized cutaneous T-cell lymphoma is associated with large reactive lymphoid infiltrates around the periphery of the lymphoma, and repeat biopsies are often necessary to obtain adequate diagnostic tissue.

Disease staging depends on the extent of cutaneous and hematologic involvement and on the presence or absence of nodal and/or visceral involvement. Because survival time is closely related to the presence or absence of extracutaneous disease, all patients should undergo a thorough workup. This includes a complete physical examination with emphasis on skin and lymph node assessment, examination of peripheral blood for mononuclear cell abnormalities, and imaging studies, including a chest x-ray. If clinical suspicion for visceral disease exists, ultrasonography and/or computed tomography can help confirm extracutaneous disease. Dissemination to lymph nodes and viscera is seen at autopsy in 71% of cases. [8]

The case we report in this article is significant for two reasons. First, it represents an unusual location for cutaneous T-cell lymphoma. Therefore, a high index of suspicion should be maintained for occult malignancies in patients who present with presumptive cutaneous infections that do not respond to antibiotic therapy. Second, it demonstrates the difficulty of making a tissue diagnosis. The reason for this might be the reactive lymphoid infiltrate located in the periphery of the lymphoma. A repeat biopsy might be necessary to make a definitive diagnosis. In our patient, an excision of the lesion was necessary to arrive at a diagnosis. Our patient was treated postoperatively with a total dose of 4,000 cGy of 12 Me V electron-beam radiation to the left auricle and ipsilateral neck. He remains free of disease 10 months following the initiation of therapy.

From the Department of Otolaryngology--Head and Neck Surgery (Dr. Baumgartner, Dr. Eusterman, and Dr. Massengill), and the Department of Pathology (Dr. Myers), Madigan Army Medical Center, Tacoma, Wash.

Reprint requests: Vincent Eusterman, MD, DDS, Chief, Department of Otolaryngology--Head and Neck Surgery, Madigan Army Medical Center, Tacoma, WA 98431. Phone: [phone]; fax: [phone]; e-mail: [e-mail]

The views expressed in this article are those of the authors and do not reflect the official policy or position of the United States Army, the Department of Defense, or the United States Government.

References

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