|LETTER TO THE EDITOR - DOCUMENTING A CASE
|Year : 2011 | Volume
| Issue : 3 | Page : 379-381
Giant axillary swelling alongwith multiple ulcers: Primary malignant adnexal tumor-A rare distinct clinicopathologic entity
Rikki Singal1, Lakshmi Narayan Garg1, Pinky Pande2, Narian S Sharma1, Bir Singh1, Deepesh Benjamin Kenwar1, Amit Mittal3, Samita Gupta3
1 Department of ENT, Maharishi Markandeshwer Institute of Medical Sciences and Research, Mullana, District-Ambala, Haryana, India
2 Department of Pathology, Maharishi Markandeshwer Institute of Medical Sciences and Research, Mullana, District-Ambala, Haryana, India
3 Department of Radiodiagnosis and Imaging, Maharishi Markandeshwer Institute of Medical Sciences and Research, Mullana, District-Ambala, Haryana, India
|Date of Web Publication||2-Nov-2011|
C/o Dr Kundan Lal Hospital, Ahmedgarh, District: Sangrur - 148021, Punjab
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Singal R, Garg LN, Pande P, Sharma NS, Singh B, Kenwar DB, Mittal A, Gupta S. Giant axillary swelling alongwith multiple ulcers: Primary malignant adnexal tumor-A rare distinct clinicopathologic entity. J Can Res Ther 2011;7:379-81
|How to cite this URL:|
Singal R, Garg LN, Pande P, Sharma NS, Singh B, Kenwar DB, Mittal A, Gupta S. Giant axillary swelling alongwith multiple ulcers: Primary malignant adnexal tumor-A rare distinct clinicopathologic entity. J Can Res Ther [serial online] 2011 [cited 2020 May 30];7:379-81. Available from: http://www.cancerjournal.net/text.asp?2011/7/3/379/87023
Sweat gland neoplasms are rare adnexal tumors represent 1-2% of skin cancers.  Clear cell hidradenoma is a lesion with histopathological features resembling those of eccrine poroma and eccrine spiradenoma which was first described by Liu in 1949 as clear cell papillary carcinoma of the skin.  A variety of names are applied to dominantly dermal-based malignant eccrine tumors, including hidradenocarcinoma, malignant acrospiroma and clear cell eccrine carcinoma. This is a group associated with aggressive biological behavior and frequent distant metastases. 
A 50-year-old male presented with a swelling in the left axilla since 3 months which was small in size and slowly increased in size to the present size of approximate 6 cm globular in shape. Fever was present off and on since 2 months. He took medicine from the private practioner but there was no relief. No other complaints were present except for discomfortness. On local examination, the swelling was present in center of the left axilla in pedenculated form, measured 6 x 6 cm in diameter and was firm in consistency, non-tender and globular in shape [Figure 1]. It was not fixed to the underlying structures. Skin over the swelling was reddish in color and multiple ulcers were present in center of the swelling from which minimal pus and bloody discharge was coming out. No axillary lymph nodes were felt. Rest of the examination was normal.
|Figure 1: A large swelling seen in the left axilla with multiple ulcers over the skin surface|
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In routine blood tests, total leukocyte counts and erythrocyte sedimentation rate were raised. Mantoux test was negative. On fine needle aspiration cytology, it came as malignant neoplasm adenocarcinoma. Ultrasonography revealed a well-defined round soft tissue lesion of size 4.43 cm in the left axilla. Lesion shows mild to moderate flow in it. Diagnosis was made as soft tissue sarcomas with few lymph nodes were present. Chest X-ray was normal.
Patient took for surgery and elliptical incision was given in left axilla. Wide resection of the swelling with clearance of the lymph nodes alongwith skin margins of 2 cm was done [Figure 2]. Skin was closed easily and a corrugated drain was kept in the axilla. On cut section, the tumor was grayish, hemorrhagic to blackish in color with variegated appearance and was friable [Figure 3]. Microscopically, the tumor was composed of clear cells and polyhedral cells. The individual tumor cells are highly pleomorphic having hyperchromatic nucleus, irregular nuclear membrane and clear cytoplasms [Figure 4] and [Figure 5]. Most of the tumor cells were showing intense PAS positivity and areas of angioinvasion was also appreciated. There were numerous mitotic figures and many atypical mitotic activities were seen. On histopathology diagnosis was made as malignant nodular hidradenoma (adnexal tumor). Patient was discharged in satisfactory condition.
|Figure 3: On gross cut section of the tumour, it appears as variegated and hemorrhagic brownish in colour|
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|Figure 4: Photomicrograph showing lobules of tumor cells separated by fi brocollagenous stroma (H&E, stain ×-40)|
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|Figure 5: Photomicrograph showing lobules of tumor cells separated by fi brocollagenous stroma [thick arrow] and abnormal mitotic fi gures [thin arrow] (H&E, ×-200)|
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Malignant tumors of the sweat glands are very rare. Hidradenoma is a benign sweat gland tumor, which usually presents as a solitary unencapsulated dermal nodule, with occasional extension into the subcutaneous fat.  Sweat gland carcinoma represents a rare group of tumors with potential for destructive local tissue infiltration and regional as well as distant metastasis. Melanoma skin cancers account for 4-7% of all skin cancers, the non-melanoma tumors for 93-96%. Both basal cell and squamous cell carcinomas represent > 80% of non-melanoma skin cancers whereas benign and malignant adnexal tumors represent only 1-2%, including mesenchymal, fatty and vascular tumors.  Hidradenocarcinoma is also often referred to as malignant nodular/clear cell hidradenoma, malignant clear cell acrospiroma, clear cell eccrine carcinoma or primary mucoepidermoid cutaneous carcinoma [Table 1]. 
Adnexal carcinomas of the skin derive from structures that have a common origin such as the apocrine and eccrine sweat glands, sebaceous glands and hair follicles.  The eccrine glands are present everywhere, except the lips, glans penis, inner surface of the prepuce, clitoris and labia minora. They are most dense on palms and soles and respond primarily to cholinergic stimuli, thereby playing an important role in regulating the body temperature. These posses no distinctive clinical features making diagnosis by gross appearance virtually impossible. They usually manifest as non-tender, subcutaneous nodules, primarily in elderly individuals.
The apocrine sweat glands are limited to ear canal, the eyelids, the axilla, the anogenital region and the mammary areola and are under the control of sexual hormones. Apocrine carcinomas manifest as non-tender single or multiple, firm, rubbery or cystic masses with red to purple overlying skin.  Division of sweat gland carcinomas into eccrine and apocrine groups, although desirable, is not clinically useful as the existing literature has not adequately subdivided and studied the separate entities well enough to make this distinction worthwhile. ,, The tumor was first described by Goldstein in 1982 and originates from pleuripotent adenexal cells capable of eccrine and follicular differentiation.  He described six neoplasms with certain common histological features: (i) cords and nests of cytologically uniform keratocytes (ii) keratotic cyst-like structures (iii) variable ductal differentiation (iv) sclerotic collagenous tissue (v) perineural invasion and (vi) no lymphatic invasion.
Sweat gland carcinomas occur in women between 60 and 70 years of age. It commonly occurs in the genital skin and perineum (34.5%), followed by trunk (26.4%), head and neck (18.3%) and lower extremities (13.9%). This carcinoma may appear as a solitary, yellowish and painless nodule. Histology shows very cellular bands or basophilic masses extended from the dermis to the subcutaneous weave. 
The criteria for malignancy include poor circumscription, presence of nuclear atypia and mitotic activity, predominantly solid cell islands, infiltrative growth pattern and angio-lymphatic permeation as seen in our case.  Microscopically they have appearance of an adenocarcinoma with well-developed glandular lumina, showing characteristic evidence of decapitation secretion. Tumor cells are PAS-positive due to glycogen granules and diastase resistant, as in our present case it was positive.  The recommended treatment of all subtypes of sweat gland carcinomas is wide surgical excision along with regional lymph node dissection in the presence of clinically positive nodes.
Malignant tumors of the sweat glands are very rare neoplasms with no discrete clinical characteristics. It is necessary to go for histological evaluation if any lesion presents as a large mass and to verify its status. Additional resection is generally required, with at least 2-cm clear margins, since surgery is the only effective treatment.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]