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Sweat gland neoplasms


Blue sweat gland tumors differentiate toward the secretory portion of the sweat gland. Red sweat gland tumors differentiate toward the sweat duct. Sweat duct tumors often demonstrate clear cell change. Most sweat gland tumors can show at least focal decapitation secretion, suggesting they are capable of apocrine differentiation.

Cylindroma (turban tumor)

Key Features

  • Islands of blue cells with little cytoplasm

  • Dark and pale blue nuclei present

  • Jigsaw-puzzle pattern

  • Islands outlined by a deeply eosinophilic basement membrane

  • Deeply eosinophilic hyaline droplets may be noted in islands

  • Often inherited and multiple on the scalp

Fig. 5.1, Cylindroma

Spiradenoma

Key Features

  • Larger round islands of blue cells with little cytoplasm

  • Dark and pale blue nuclei present

  • Islands peppered with black lymphocytes

  • Deeply eosinophilic hyaline droplets may be noted in islands

  • Usually solitary

Spiradenomas and cylindromas are closely related tumors. Both differentiate toward the secretory portion of the sweat gland. Hybrid tumors occur. Spiradenomas are usually sporadic and solitary, whereas cylindromas are multiple, inherited, and may occur together with trichoepitheliomas. Spiradenomas are inflamed (lymphocytes) and spontaneously tender.

Fig. 5.2, Spiradenoma

Table 5.1
Characteristics of cylindroma versus spiradenoma
Characteristic Cylindroma Spiradenoma
Blue cells with little cytoplasm Yes Yes
Dark and pale blue nuclei Yes Yes
Peppered with black lymphocytes No Yes
Hyaline droplets in nests Yes Yes
Jigsaw-puzzle pattern Yes No
Deeply eosinophilic basement membrane Yes No
Inheritance Autosomal dominant Sporadic
Tender No Yes

Pearl

Tender tumors: BANGLE

  • B lue rubber bleb nevus

  • A ngiolipoma

  • N euroma, neurilemmoma

  • G lomus tumor

  • L eiomyoma

  • “ E ccrine” spiradenoma

These tumors are probably tender because they have:

  • 1

    Smooth muscle that can contract to cause pain

  • 2

    Compressed nerve

  • 3

    Inflammation

Spiradenocarcinoma

Key Features

  • Rare

  • Occurs in long-standing spiradenomas

  • Atypia, mitoses, and necrosis

Fig. 5.3, Spiradenocarcinoma

Syringocystadenoma papilliferum

Key Features

  • Opens to surface

  • Blue papillary fronds extending upward into clear spaces: “fjords and fronds”

  • Decapitation secretion

  • Plasma cells

Syringocystadenoma papilliform (SPAP) differentiates toward the secretory portion of the sweat gland, and S PAP opens to the s urface, which looks as though one could s lide into it. Plasma cells are typically present in the mesenchymal core of each frond. Clinically, they appear as raised warty plaques on the head or neck. One third of cases occur within nevus sebaceus.

Fig. 5.4, Syringocystadenoma papilliferum

Hidradenoma papilliferum

Key Features

  • Blue dermal nodule with branching cystic spaces

  • Papillary fronds

  • Decapitation secretion

Hidradenoma papilliferum (HPAP) usually presents clinically as a vulvar dermal nodule, but occasionally presents on a breast, eyelid, or ear. Histologically, H PAP is a mazelike dermal nodule, which looks as though one could h ide in it. The arborizing pattern of blue fronds demonstrates decapitation secretion. Like SPAP, it differentiates toward the secretory segment.

Fig. 5.5, Hidradenoma papilliferum

Papillary digital carcinoma (aggressive digital papillary adenocarcinoma)

Key Features

  • Blue tumor nodules with cystic change

  • Little to no visible cytoplasm

  • Papillary fronds

  • Atypia, mitoses, and necrosis variable

  • Typically involves the hand

  • Many patients are young

  • Even bland tumors can metastasize

There can be significant histologic similarities to HPAP. All digital papillary tumors should be considered carcinomas.

Fig. 5.6, Papillary digital carcinoma

Mucinous carcinoma

Key Features

  • Islands of blue cells surrounded by mucin (“blue islands floating in a sea of snot”)

  • Primary cutaneous mucinous carcinoma and metastatic mucinous carcinoma look identical

Mucinous carcinoma can be primary in the skin or can be metastatic from a primary cancer of the breast or gastrointestinal tract. Imaging studies may be required

Fig. 5.7, Mucinous carcinoma

Syringoma

Key Features

  • Paisley-tie pattern of tadpole-shaped ducts

  • Ample pink cytoplasm

  • Dense red sclerotic stroma

  • Tumor is small and round

Syrinx refers to a pipe or duct. Syringomas usually appear as small papules on the eyelids. They are especially common in Asian women and in children with Down syndrome. Eruptive syringomas typically occur on the chest, back, or penis of a dark-skinned patient. Eruptive syringomas appear as small hyperpigmented papules with no tendency to coalesce.

Fig. 5.8, Syringoma

Clear cell syringoma

Key Features

  • Paisley-tie pattern of tadpole-shaped ducts

  • Clear cells containing abundant glycogen

  • Dense red sclerotic stroma

  • Tumor is small and round

  • Associated with diabetes mellitus

Fig. 5.9, Clear cell syringoma

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