Sebaceous gland carcinoma C44.L4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 23.04.2021

Synonym(s)

adenocarcinoma sebaceum; Carcinoma, sebaceous gland carcinoma; carcinoma sebaceum; Sebaceous adenocarcinoma; sebaceous carcinoma; Sebaceous gland carcinoma

Definition
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Rare carcinoma of the adnexa with differentiation of sebaceous glands. Occurs on the skin also in the context of Muir-Torre syndrome.

Classification
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A distinction is made according to localization:

  • Carcinoma of the sebaceous glands localized ocularly
  • Extraocularly localized sebaceous gland carcinoma

Manifestation
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Isolated on the skin predominantly occurring from the age of 70 onwards. In the eye and in the Torre-Muir syndrome, occurrence is possible as early as adolescence.

Localization
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Mostly in the periorbital region, much more rarely nose, eyebrow, ear, lip, temple. In individual cases also localised on the trunk. The occurrence at the oral mucosa, vulva and in the parotis has also been described.

Clinical features
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0.5-2.0 centimetre lump. The clinical picture is uncharacteristic and indistinguishable from other malignant non-melanoma tumours of the skin. Mostly appearing as crusty or ulcerated, red-yellow nodule or correspondingly coloured plaque. Single tumors impress under the picture of a keratoacanthoma. Occurrence also described in Muir-Torre syndrome and in sebaceous nevus.

Histology
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Asymmetric, infiltrating tumour with a broad morphological differentiation spectrum from relatively mature cells (pattern dominated by sebocytes) to immature cells (basaloid cell type, hardly mature sebocytes; numerous mitoses). The tumor strands are homogeneous, compact, composed of basaloid cells with cell and nuclear atypia and numerous mitoses. In addition, there are also mature cellular parts with adenoid structural pattern. Nests of mature sebocytes may also occur in these areas. Sebaceous gland carcinomas may infiltrate the epidermis pagetoid. In unclear tumors, the EMA immunoperoxidase technique (epithelial membrane antigen) provides good evidence of sebocytic differentiation.

Differential diagnosis
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Therapy
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The treatment of ocular sebaceous gland carcinomas belongs in the hands of an experienced ophthalmologist. Otherwise, complete surgical excision (see also spinocellular carcinoma) of the primary tumour with a safety margin of at least 1 cm. Edge incision controls necessary. A sentinel lymph node dissection (SNLD) should also be performed.

Extraocular: Complete surgical excision of the primary tumor with a safety margin of 1 cm.

S.a.u. carcinoma, spinocellular. Sonographic and histological evaluation.

Progression/forecast
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Metastasis usually occurs 1-2 years after the tumor has appeared, initially in the regional lymph nodes. Later, visceral metastases may also occur.

Note(s)
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Significant metastatic potential. Ocular sebaceous gland carcinomas metastasize or recur in about 30% of cases. The metastasis rate of extraocular tumours is not certain, but lower than that of ocular tumours.

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 23.04.2021