Volume 3, Issue 1 , Pages 24-27, January 2008
Eccrine acrospiroma of neck in a 3-year-old female child
Article Outline
Summary
Eccrine acrospiromas are tumours derived from eccrine sweat gland epithelium. They are usually found in middle-aged people with equal sex predilection. Most of such tumours are found on extremities. We present a case of 3-year-old female child who presented to our institute with a swelling in neck just above the medial end of right clavicle. Thorough clinical, laboratory and radiological investigations were carried out. Taking into consideration the age of the patient and other clinical features a diagnosis of sebaceous cyst of neck was arrived at. The mass was excised under general anaesthesia and sent for histopathological examination. The pathological report established a diagnosis of benign eccrine acrospiroma. The patient has no complaint postoperatively and has been advised to be on regular follow up due to reported cases of recurrence and malignant transformation. Such case of eccrine acrospiroma is rare and has never been reported in the literature and thus should be kept in mind while dealing with neck swellings especially in children taking into consideration its malignant potential.
Keywords: Eccrine acrospiroma, Hidradenoma, Clear cell hidradenoma, Sebaceous cyst, Poroma, Child, Neck, Malignant eccrine acrospiroma, Epidemiology
1. Introduction
Acrospiroma occurs as a single mass in the skin and is nodular and solid or cystic. The colour varies from that of the surrounding skin to red or reddish blue, and the covering skin may be smooth or thickened and verrucous. The tumours are rarely painful, and a serous or haemorrhagic fluid may drain spontaneously from them. They may recur but rarely undergo malignant change. Clinically, the tumours lack diagnostic specificity, but they may be included in the differential diagnosis of nodular and cystic lesions of the skin. Histologically, the acrospiroma is readily differentiated from other sweat gland tumours [1]. Usual location of this tumour (as the name implies) is acral i.e. the extremities. We present a case of acrospiroma in neck in a 3-year-old female child.
2. Case summary
A 3-year-old female child presented to our ENT OPD with chief complaint of swelling neck just above the medial end of right clavicle since 4 months. The swelling was first noticed 4 months back by her mother when she was bathing her. Initially, it was the size of a pea. In due course it progressively increased and was at the time of presentation of the size of one rupee coin (Indian currency roughly measuring 2.5
cm in diameter). There was no diurnal variation in the size of the swelling. There was no change in size of swelling with posture or on deglutition.
There was history of pain in the swelling off and on. The pain usually lasted for a few minutes and subsided by itself without any medication or intervention. There was no history of any discharge from the swelling There was no history of any fever or difficulty in swallowing.
There was not any relevant past or family history.
On examination the patient had a smooth, reddish blue, swelling on the neck just above the medial end of right clavicle [Fig. 1]. The most prominent part of the swelling had rough skin but did not have any punctum or ulcer formation. The swelling was rounded, 2cm×1cm in size, cystic to feel, freely mobile vertically and horizontally. It was not attached to underlying structures and did not move with deglutition.
Fig. 1.
Preoperative photograph of the patient at medial end of clavicle (right side) that finally proved to be an eccrine acrospiroma.
Routine laboratory investigations (haemoglobin, total and differential cell count, bleeding and clotting time, complete urine examination) were carried out which were within normal limits. Chest X-ray was normal.
Ultrasonography of the swelling showed 15mm×8mm cystic swelling.
A provisional diagnosis of sebaceous cyst was arrived at and excision biopsy was planned under general anaesthesia. After excision of the mass it was sent for histopathological examination in 10% formalin.
Postoperative period was uneventful without any complications.
The report of the biopsy showed histological picture suggestive of eccrine acrospiroma [Fig. 2]. Microscopically, the skin-covered tissue showed sharply demarcated lobules in the dermis. The cells were arranged in intervening cords. The tumour was made up of two types of cells, one with small dark nuclei with clear cytoplasm and others with large pale nuclei. Hyaline material was focally present in stroma.
Fig. 2.
Histopathological picture showing the excised mass to be eccrine acrospiroma.
After the report patient is now on regular follow up and has been advised to watch out for any recurrence at the same or any other site. Her parents have been explained the benign nature of the disease and potential chances of any recurrence or malignant change.
3. Discussion
Epithelial tumours of the cutaneous adnexae are considered to originate from three different structures, i.e., hair follicles, eccrine/apocrine sweat glands, and sebaceous glands. Although this derivation provides a practical basis for classification, some tumours may exhibit a mixed differentiation and thus should be categorized according to the prevailing phenotype [2]. These tumours generally behave in a benign manner, but malignant types exist. Most adnexal tumours are not common enough to permit most practitioners to maintain a ready familiarity with them, but they also are not rare [3].
Hidradenoma (eccrine acrospiroma) is a generally benign, dermal appendage tumour that usually presents as a solitary unencapsulated dermal nodule, with occasional extension into the subcutaneous fat. There is some disagreement as to the differentiation of hidradenoma (eccrine acrospiroma), but it has been regarded as an eccrine sweat gland tumour on the basis of enzyme, histochemical and electron microscopic features, which include microvillus processes, abundant glycogen granules, and numerous mitochondria. Clear cell hidradenoma is a variant of hidradenoma with several other designations, including clear cell myoepithelioma, nodular hidradenoma, eccrine sweat gland, adenoma of clear cell type, solid cystic hidradenoma and eccrine acrospiroma [4].
Benign eccrine poromas and acrospiromas are related tumours with similar cytologic features. Both contain small squamoid cells that resemble those of seborrheic keratosis. A poroma is a superficial tumour confined to the epidermis and upper dermis. An eccrine acrospiroma is a deep dermal or subcutaneous nodular neoplasm that is sometimes connected to the epidermis [5].
There is a dearth of the literature on poroid neoplasms therefore a generalization of its epidemiology is uncalled for. But recently two series—one of 166 cases by Yakoob et al. from Pakistan [6] and one of 25 cases by Chen et al. from Taiwan [7] may suggest that they are more common in Asian continent.
Clear cell hidradenoma (eccrine acrospiroma) presents as a solitary, firm nodule with a slight predilection for the head, face, and upper extremities. A review of nodular hidradenomas by Hernandez-Perez and Cestoni-Parducci revealed a female predominance (1.7:1), with a mean age at presentation of 37.2 years. Sites of involvement were the head (30.3%), upper limb (25.8%), and trunk (20.2%). In the largest series presented in the literature by Abenoza and Ackerman, of 353 poromas 30% were located on face, 10% on scalp, 14% on trunk, 15% on foot and 5% on hand [8]. In a study by Yakoob et al. majority of eccrine acrospiromas were in the age range of 21–50 years with not even a single case reported in the age group presented by us [6].
The overlying skin is generally intact, although ulceration with leakage of serous fluid may be seen. Histologically, eccrine acrospiroma is a well-circumscribed dermal tumour with a grenz zone between the tumour and the epidermis. Cytologically, hidradenomas (acrospiromas) are composed of two types of cells, the proportion of which varies a great deal between tumours. One cell type is polyhedral with a rounded nucleus and slightly basophilic cytoplasm. The second cell type is generally round with clear cytoplasm [4].
Although regarded as benign, nodular hidradenoma (eccrine acrospiroma) can recur after inadequate excision. Malignant transformation of clear cell hidradenoma (eccrine acrospiroma) is rare; in one review, 6.7% of clear cell hidradenomas were malignant and were histologically characterized by nuclear atypia, necrosis, and abnormal mitoses. Reports in the literature consistently emphasize the slow growth rate of both malignant and benign eccrine acrospiromas. Although malignant eccrine acrospiromas usually arise de novo, malignant transformation in long-standing lesions can occur. Hunt et al. reported a case of malignant eccrine acrospiroma that had been present for 40 years, which strongly suggests a malignant transformation of a pre-existing benign tumour [9]. Holden et al. presented a case in which the tumour had been present for 20 years, and the fact that his biopsy specimen yielded histologic evidence of both benign and malignant components again suggested a malignant transformation [5]. In the series by Abenoza and Ackerman porocarcinoma was found adjacent to poromas in less than 1% cases [8]. An aggressive course with widely disseminated disease and death has been reported [4].
4. Summary
Eccrine acrospiroma of neck is a relatively rare tumour with a malignant potential. Possibility of the entity should be kept in mind when dealing with swelling of the neck especially in children, as it has not been reported in children before this article.
References
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- . Benign adnexal skin tumors. Pathologe. 2002;23(1):71–78
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- . Clear cell hidradenoma: a mimic of metastatic clear cell tumors. Arch. Pathol. Lab. Med. 2005;129(5):e113–e116
- . Malignant eccrine acrospiroma with metastasis to the parotid. Ear Nose Throat J. 2002;81(Part 5):352–355
- . Spectrum of cutaneous appendage tumors at Aga Khan University Hospital. J. Pak. Med. Assoc. 2003;53(9):427–432
- . Clinical and histological characteristics of poroid neoplasms: a study of 25 cases in Taiwan. Int. J. Dermatol. 2006;45(6):722–727
- . Neoplasms with Eccrine Differentiation. Philadelphia: Lea and Febiger; 1990;pp. 113–185
- . Giant ecerine acrospiroma. J. Am. Acad. Dermatol. 1990;23:663–668
PII: S1871-4048(07)00073-1
doi:10.1016/j.pedex.2007.09.002
© 2007 Elsevier Ireland Ltd. All rights reserved.
Volume 3, Issue 1 , Pages 24-27, January 2008
