Syringomas

The following describes some of the more common and selected uncommon skin tumors that may occur. The discussion will include benign tumors of the surface epidermis; premalignant lesions of the epidermis; malignant tumors of the surface epidermis; benign and malignant appendage tumors (follicular, sebaceous, and apocrine differentiation); metastatic skin tumors; tumors of fibrous, vascular, smooth muscle, fat and nervous tissue; pigmented cell lesions, including melanoma, and tumors of the lymphoid, mono-- nuclear, and hematapoietic systems.

BENIGN TUMORS OF THE SURFACE EPIDERMIS Wart (verruca)

Warts are papillomatous tumors caused by various forms of a papovavirus. The 4 types (vulgaris, plantaris, plana, and condyloma acuminatum) vary according to morphology and location. The virus causing genital warts is believed to be immunologically distinctly from the virus causing other types. The common wart (verruca vulgaris) is a raised, compact lesion with a hyperkeratotic and papillomatous surface. Lesions can be anywhere but are most common on the hands, and may be solitary or multiple. Lesions are not related to sun exposure, but may possibly be related to trauma. Treatment is variable and spontaneous regression quite common.

Seborrheic Keratoses

Seborrheic keratoses are common papillomatous growths appearing during middle age. They are circumscribed, raised lesions with a friable, verucous surface. Hyperpigmentation may occur and the lesions resemble melanoma. They are not sun related, but may be present in great numbers on the chest and back. The disease may be familial. Simple removal is the treatment of choice; they are not considered premalignant lesions.

Acanthosis Nigricans

Brownish verrucous areas, common in the neck area, characterize Acanthosis nigrans. The benign form occurs at birth or during childhood; other cases occur with obesity, endocrine, or congenital syndromes. The lesions are light colored enough so as not to be confused with malignant melanoma, and are usually larger than the latter at presentation.

Keratocanthoma

This interesting lesion is considered by some to be a variant of a well-differentiated squamous cell carcinoma (SCC). It is also called molluscum sebaceum, and is composed of proliferating keratinocytes. Typical lesions are fleshy nodules, with a central hyerpkeratotic crater. They can appear in areas not commonly involved with SCC, such as the dorsum of the finger. Most lesions reach full size in a few weeks, and then regress spontaneously in a few months, leaving a small-depressed scar. Because of this behavior, often no treatment is necessary.

PREMALIGNANT LESIONS OF THE EPIDERMIS Actinic (Solar) Keratoses

These are lesions evolving from the cumulative effect of ultra violet radiation, occurring only in sun-exposed areas, with a middle age onset. Lesions vary in appearance, characterized by focal hyperkeratosis, with associated erythema. Lesions appear as keratocytic atypia of the lower layers of the epidermis. Four types are recognized: typical, hypertrophic, acantholytic, and lichenoid. Surrounding skin usually shows solar damage, with macular foci of erythema and scaling. Solar keratoses may gradually undergo transformation to squamous cell carcinoma with low metastatic potential.

Bowen's Disease

Bowen's Disease is characterized by erythematous plaques with nodular and crusted areas; it is SCC in situ. The lesions may resemble superficial basal cell carcinoma (BCC) but differ in the absence of a thread-like pearly border. It is not related to sun damage, unlike actinic keratoses. The criterion is full thickness keratinocytic atypia in the epidermis. There is a "windblown" appearance of the epidermis, due to absence of orderly, maturation of the keratinocytes into a flattened squame. Like solar keratosis, Bowen's Disease may gradually undergo transformation into invasive SCC.

Leukoplakia

Leukoplakia refers to a raised white lesion on mucous membranes or modified mucous membrane, considered to be a precursor to oral vestibule or oral cavity carcinoma. Oral pathologists use this term on a clinical basis, referring to any white raised lesion in mucous membranes, with or without premalignant changes. On the skin, usual location is the lower lip. Both smoking and mechanical irritation, eg, a pipe stem brought repeatedly to the mouth, have been implicated as possible factors in the etiology of leukoplakia.

MALIGNANT TUMORS OF THE SURFACE EPIDERMIS Squamous Cell Carcinoma (SCC)

SCC may arise de novo or be preceded by premalignant lesions, such as actinic keratosis, radiation keratosis, or Bowen's Disease. The disease may also develop at the margins of chronic ulcers, in burn scars, or as a late sequalae of lupus erythematosus. There are individuals who have hereditary disorders which predispose them to the damaging effects of sunlight, such as albinism and xeroderma pigmentosum; these people have a high incidence of SCC. The vast majority of chemical carcinogenesis is SCC.

The tumor cells are derived from keratinocytes, and display increased cell volume, increased nuclear/cytoplasmic ratio, and decreased mitochondria, Golgi, and RER. On ultra structure, the distinguishing characteristics are the presence of cytoplasmic keratin filaments and desmosomes. Villous projections from the SCC will penetrate the basement membrane of the cells. The lesions vary tremendously in their appearance. They may be ulcers with or without raised margins, or verrucous or papillomatous in nature. They can be fleshy lesions with a hyperkeratotic center resembling keratocanthoma. Metastases are primarily through the lymphatic system to regional lymph nodes; metastases are more common from poorly differentiated tumors. Metastases can be quite invasive, infiltrating the supportive connective tissue. Treatment is by curretage, freezing with liquid nitrogen, or surgery involving the Mohs technique.

Basal Cell Carcinoma

BCC , like SCC develops in sun exposed areas and is the most common form of cancer occurring in humans. There are close to 1,000,000 cases annually in the United States. The tumor never occurs on mucosal surfaces, and is rare on plantar or palmer skin. It is believed to be a neoplasm of undifferentiated epidermal pluripotent cells; it appears to arise de novo from follicular epithelium. BCCs are believed to be "stromal dependent" thus they rarely metastasize (

Nevoid Basal Cell Epithelioma Syndrome

This is a dominantly inherited syndrome, consisting of multiple BCCs occurring at an early age, palmer and plantar pitting (pathognomonic for this syndrome), and curiously calcification of the falx cerebri. Odontogenic keratocysts of the mandible are also common, necessitating surgical removal. Cutaneous lesions in this syndrome are pearly papules and nodules with a tendency to ulcerate. There are variants; linear basal cell nevus is present at birth, and the follicular basal cell nevus is manifest in enlarging areas of hair loss in which BCC may develop. Treatment is the same as for BCC in general, but careful vigilance and frequent dermatologic examination to detect tumors early are paramount.

Mohs Surgical Technique

This technique, first described in 1936, has become widespread for surgical removal of skin tumors. The procedure involves microscopic examination of the entire interface between the defect and the tissue removed, including all peripheral margins, during the time of surgery. Horizontal shaving of the tumor is performed. The procedure is continued until the wound is free of tumor cells. Since each frozen section takes 30 minute to an hour, the procedure can be lengthy, its only major disadvantage.

BENIGN AND MALIGNANT APPENDAGE TUMORS

Epidermal Cyst, Milia, and Pilar Tumors

Epidermal cysts are common on the face, they are also called wens; they are lined with stratified squamous epithelium. Milia are yellowish white globoid superficial lesions of 1-2 mm. They are miniature epidermal cysts. Pilar tumors are large solitary or clustered nodules, or verrucous masses, most common in women and occur on the scalp.

Tricoepithelioma

This is a benign follicular tumor characterized by the triad of basaloid tumor islands, keratin cysts, and a fibroblastic stroma. The nasolabial fold is a common location for these tumors. They are flesh colored, rather then red, which distinguishes them from tuberous sclerosis.

Sebaceous Gland Tumors

These are lesions which differentiate in the direction of sebaceous glands. There are varying types and associated syndromes. Sebaceous adenomas and carcinomas may be regarded as analogues of BCC with differentiation toward sebaceous gland structures. Carcinomas in this family usually develop from the meiobian glands of the eyelids and are uncommon.

Tumors of Apocrine Differentiation

There is a widely held view that appendage tumors develop from pluripotential cells. There are many examples. Cylindromas can be solitary or multiple, and usually involve the face or scalp. Individual lesions are potato like in appearance; large tumors of the scalp are called turban tumors. Paget's Disease is a disorder of the female nipple and areola, characterized by induration, scaling, and erythema. Apocrine adenocarcinoma is uncommon. Syringomas are actually of eccrine lineage; they affect women more than men. Lesions are small papules and nodules occurring in the skin of the eyelids and periorbital area. They appear during puberty or adult life.

METASTATIC SKIN TUMORS

Metastatic skin tumors most commonly arise from breast or lung primaries, the former origin being the most common. Inflammatory carcinoma of the breast, with peau de orange skin changes, is in this category. The tumors may appear as discrete nodules or skin induration and infiltration. Skin metastases from breast cancer can become extensive and are very distressing. Metastatic tumors to the skin from other primaries are uncommon but any primary cancer can be causative. Treatment is palliative; whirlpool for large involved areas can be soothing and comforting to the patient and may be indicated in selected cases.

CONNECTIVE TISSUE TUMORS

Fibrous Tissue

Dermatofibromas are indurated nodules most frequently located on the extremities. Histiocytes may be present in the lesion. Skin tags are soft pednuculated tumors. Most are removed for cosmetic reasons. Dermatofibrosarcoma protuberans is an uncommon but interesting lesion, occurring most commonly on the scapular region. It begins as an intracutaneous plaque, with bluish or reddish nodules. Locally invasive and ulcerative, it commonly recurs after surgical removal. Muscle flaps and skin grafts may be necessary in definitive surgery.

VASCULAR TUMORS

Benign Tumors

These tumors vary in appearance and nomenclature. Spider nevi consist of papules with reddish branching capillaries. Port wine stain (nevus flammeus) is common at birth; large lesions may be unsightly but otherwise innocuous. Some spontaneously regress. The Klippel Traunaunay Syndrome consists of multiple nevus flammueus with hypertrophy of soft tissue and bone, with resultant physical therapy issues. Cherry hemangiomas have an onset in mid life and are usually multiple, on the trunk. Glomus tumors are raised, violaceous lesions most commonly on the distal areas of the digits, and may be painful. Lymphangiomas may be clusters of translucent papules, or deep boggy lesions that may produce deformity. Lesions in the neck or groin are called cystic hygromas.

Kaposi's Sarcoma

Kaposi's sarcoma (KS) in its classical form occurs in elderly Jewish and Italian males, and consists of multiple infiltrated plaques and nodules of the lower extremities, with associated edema. The disease is slowly progressive. The association with HIV infection was noticed in 1981, when 45-50% of male homosexuals with AIDS developed the disease. Today, 15% of all patients with HIV develop the disease during the course of AIDS. The reason for the decreased incidence over the last 20 years is believed to be the lessened transmission (due to safer sex) of the viral agent which may be the cause of KS. KS is suspected to be the diff-use vascular proliferative response to an infectious agent, due to its occurrence in a well defined population. Treatment is variable; radiation may be used for large lesions, and topical chemotherapy is also employed.

Angiosarcoma and Lymphangiosarcoma

Angiosarcomas are rare malignant tumors of blood vessels. They occur in elderly individuals, usually in the head and neck area. It spreads through the blood stream or lymphatics. Lymphangiosarcoma usually occurs in conjunction with the Stewart Treves Syndrome, which is malignant degeneration of the lymph vessels in chronically lymphedematous extremities. The interval from mastectomy and edema to the syndrome may be as long as 20 years.

SMOOTH MUSCLE, FAT, AND NERVE TUMORS

Leiomyomas arise from smooth muscle and can be solitary or multiple. Lipomas are soft subcutaneous tumors, occurring most commonly on the trunk, neck, and forearms. Liposarcomas rarely develop in the subcutaneous fat, but rather in fascial planes between muscles often in the retroperitoneum. Metastases may occur in the skin. Neuromas represent regenerative responses rather than true tumors. Neurilemmomas are solitary soft lesions developing along a nerve. Malignant transformation is rare except in individuals with neurofibromatosis.

PIGMENT CELL LESIONS

Benign Lesions

Freckles are ubiquitous, and consist of hyperpigmented macules. A junction nevus usually has uniform pigmentation. Compound nevi are raised and papular. Congenital nevi may be hairy (hairy nevus). A halo nevus is of comment because of the zone of hypopigmentation that manifests as the nevus regresses.

Malignant Melanoma

The incidence of malignant melanoma (often just simply called melanoma) is increasing and cases are being seen at a younger age. Lesions may arise in pre-existing benign nevi, or may arise de novo. Lentigo melanoma refers to melanoma occurring usually on the face or sun exposed areas, remaining superficial and spreading peripherally with uneven pigmentation. The lesions remain local and do not metastasize. Superficial spreading melanoma resembles lentigo melanoma, but the borders are usually more circumscribed. Lesions are usually smaller than lentigo melanoma. Nodular melanoma presents as a raised lesion from the beginning; these tumors may reach considerable size quickly. They are the most dangerous form of melanoma, since they invade deeper tissues at an early stage. Most reports indicate that it is the depth of penetration of the lesion, and not the size, that determines prognosis.

Metastatic melanoma is common, and skin metastases are not unusual. These metastatic lesions take on a variety of appearances; satellitosis (in transit lymphatic metastases) are commonly seen. In transit and retrograde lymphatic metastases may ulcerate. Lymph node dissection either performed prophylactically or for metastases, particularly of lesions in the lower extremity, can cause lymphedema, and CLT is indicated when this ensues. In fact lymphedema prevention is paramount and efforts should be organized in this direction.

LYMPHOID, MONONUCLEAR, AND HEMATOPOIETIC TUMORS OF THE SKIN

Lynthoma Cutis

Lymphoma of the skin can be classified in a variety of ways, and the classification system changes frequently dependent on recognition of subcategories of lymphoma. Primary lymphoma of the skin is uncommon, considering the incidence of Hodgkin's and non-Hodgkin's lymphoma in general. Cutaneous lesions may be the primary tumor of lymphoma, others believe that the disease is systemic from the onset. Cutaneous lesions of lymphoma are nodules or plaques that may be flesh colored, brown, or violaceous. Ulceration as the tumor enlarges is common. If the diffusion of tumors cells is widespread in the skin, exfoliative dermatitis may develop. Pruritis may be an early symptom. Herpes zoster is common in cutaneous lymphoma, either a result of anergy of the disease itself or as a result of immunosuppressive therapy. Burkitt's lymphoma is a B cell neoplasm, occurring endemically in Africa with a predilection for the mandible. There is evidence of a viral etiology with association with the Epstein-Barr virus.

Mycosis Fungoides

Of all skin cancers that the physical therapist may encounter, mycosis fungoides (MF) is one of the most striking disorders, causing long standing morbidity. The name is a misnomer itself, as it is not a mycotic infection. MF is named for the mushroom like tumors that occur in terminal stages. It is a slowly progressive polymorphous T cell lymphoma that begins in the skin and is systemic in nature from its inception. The cells appear in tissue stains as mononuclear cells with hyperchromatic nuclei. The onset is in the fifth decade or later. Early lesions resemble a variety of skin disorders, such as psoriasis, dermatitis, lupus erythematosus, and others. The malignant cells are often not present in early stages, so diagnosis is difficult until the disease is well established. Three stages are recognized. In the premycotic stage, the lesions are erythematous, slightly raised, scaling lesions that may be annular. Pruritis is intense but is not pathognomonic as many skin diseases cause itching. This stage lasts a varying number of years, and ultraviolet therapy may assist in resolving lesions. The plaque stage is characterized by brownish plaques of varying sizes, that may be scaling and ulcerative. Lesions here too may be annular with central clearing. The tumor stage displays mushroom like tumors of varying sizes, and ulceration is typical. Topical and/or systemic chemotherapy may help to delay disease progression but the majority of patients succumb to internal spread or septicemia. Hydrotherapy in the plaque and especially the tumor stage can be beneficial, and meticulous skin care and treatment require specialized management. Cases treated in major cancer centers offer the best hope of successful palliation, particularly in late stages.

Sezary Syndrome

The Sezary syndrome occurs when leukemia infiltrates the skin, or as a terminal event in ME It is characterized by exfoliative dermatitis, generalized lyphadenopathy, and T cell leukemia. Hepatosplenomegaly is also common. Treatment is palliative, and the prognosis is poor.

Other Cutaneous Lymphomas

Steve Gudas, PT, PhD Cancer Rehabilitation Virginia Commonwealth University Medical College of Virginia Richmond, Virginia

Copyright Rehabilitation in Oncology 2001
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