apocrine carcinoma , also known as apocrine - gland carcinoma and sweat - gland carcinoma comprises of a group of extremely rare , malignant tumor , which shows features of apocrine differentiation .
the disease is primarily diagnosed in the fifth to seventh decade of life , with similar incidence in men and women and without racial predilection .
the malignancy arises at the sites of apocrine glands , which have a relatively limited distribution in the body and are found in the axillae , the medial aspect of upper arm , areola , lateral aspects of the breasts , ear canals , eyelids , and anogenital region .
patients usually present with a slow - growing , purple or red skin mass , which can be either firm or cystic in consistency .
morphologically , the tumor is an adenocarcinoma with varying degrees of differentiation composed of cells with eosinophilic cytoplasm .
characteristic histopathologic findings include decapitation secretion , a feature considered pathognomonic for apocrine differentiation , periodic - acid - schiff - positive material in the cells or lumen , and immunoreactivity with gross cystic disease fluid protein .
normal apocrine glands and apocrine - gland adenomas are often found alongside the tumor and are occasionally infiltrated by carcinoma cells .
apocrine - gland carcinoma spreads via both lymphatic and vascular routes , with metastatic disease found in regional lymph nodes , lungs , liver , and bone .
approximately one - third of patients have regional lymph node involvement at diagnosis ; the incidence is higher in patients with higher grade tumors . the female breast can be conceived developmentally and morphologically as a modified apocrine gland and therefore breast carcinoma with apocrine features can be indistinguishable from cutaneous ac .
breast parenchymal malignancy is more common than skin adnexal carcinoma , and therefore in a woman with a history of breast carcinoma it is customary to consider such a skin neoplasm the result of secondary spread unless it is possible to prove otherwise .
the only curative therapy for localized apocrine - gland carcinoma involves wide local excision with regional lymph node dissection and consideration of postoperative radiotherapy in patients with moderately or poorly differentiated tumors .
early diagnosis is , therefore , critical , and all patients with enlarging masses of unknown etiology in areas of apocrine glands should have excisional biopsies .
the clinical course is characterized by a high incidence of local recurrence ( 28% in one report ) , which is often managed by resection and radiation therapy .
however , no standard guidelines exist regarding appropriate schedules and doses due to the rarity of the diagnosis .
metastatic disease to the lung , skin , bone , brain , and kidney has been described and the disease is invariably fatal at this stage .
a 62-year - old asian male presented to a satellite hospital with the complaint of excessive tears for more than five years and left eye swelling for two months .
initially , swelling was limited to the left eyelid but progressed rapidly to size of a tennis ball .
orbital computed tomography ( ct ) scan demonstrated a localized left orbital mass with suspicion for a tumor .
the biopsy revealed poorly differentiated adenocarcinoma with polygonal tumor cells with large , hyper - chromatic nuclei , prominent nucleoli and abundant eosinophilic cytoplasm ( figures 12 ) .
the pathognomic findings of the decapitation secretion ( figure 1 ) and immunoreactivity with gross cystic disease fluid protein ( figure 2 ) supported the diagnosis of apocrine adenocarcinoma of skin . 

figure 1skin with infiltrating tumor composed of complex glandular structures within dermis ( hematoxylin and eosin staining ) .
skin with infiltrating tumor composed of complex glandular structures within dermis ( hematoxylin and eosin staining ) . 

he presented again after several months with progressively increasing facial deformity and inability to open his left eye .
the physical examination revealed significant periorbital erythema with significant sero - sanguineous discharge from the skin lesion .
metastatic survey with a bone scan unfortunately revealed multiple areas of uptake throughout the left orbital area .
ct head and neck demonstrated a solid mass lesion in the medial part of left lower eyelid and enlarged cervical lymph nodes ( figure 3 ) .
non - surgical treatment including chemotherapy and radiotherapy was discussed in detail with the patient and he eventually agreed to chemotherapy .
the patient received cisplatinum 50 mg / m , adriamycin 50 mg / m , and cyclophosphamide 500 mg / m .
computed tomography scan showing infiltration of tumor . a follow - up bone scan and ct scan of
the patient clinically had progressive proptosis and worsening soft tissue thickening on the left side of his face .
the patient was eventually referred to our service for consideration of enrollment in an experimental clinical trial .
the patient was treated on several phase i experimental protocols , but eventually had persistent thrombocytopenia which precluded him from pursuing any additional therapy .
the patient s disease started to cross the midline of the face and ac developed in his right eyelid as well .
the right eye lid became quickly and progressively swollen causing his right eye to shut .
unfortunately , the patient became incapacitated , as he was not able to see out of both eyes ; in essence the disease had rendered him blind .
ac also was present on his lips and perioral area causing difficulty with eating and swallowing food and liquids .
six years after his initial diagnosis , the patient entered hospice for supportive and palliative care .
case reports have described transient responses to various chemotherapy agents such as , vincristine , bleomycin , cyclophosphamide and doxorubicin .
described a patient with widely metastatic apocrine - gland carcinoma of the eyelid with an excellent response to systemic 5-fluorouracil chemotherapy .
mezger j. , et al . described two patients with sweat - gland carcinoma who responded to combination chemotherapy with doxorubicin , cyclophosphamide , vincristine , and bleomycin . in one of these patients , a complete remission of two years
duration was achieved ; the other patient had a partial remission of four months duration .
the prognostic factors for sweat gland carcinoma are difficult to identify , again owing to the small number of reported cases .
clinical trials of therapy , although difficult to conduct in a disease of low prevalence , provide additional options for patients and physicians .
this case illustrates the detailed diagnostic evaluation and the need for high suspicion by the primary physicians to consider apocrine carcinoma of skin as a differential diagnosis for a skin lesion because of the high propensity of this tumor to locally metastasize and the lack of evidence - based clinical guidelines in literature for its management due to the rarity of the tumor .
patients should be highly encouraged to undergo surgical resection , as this often is the only modality of therapy with curative intent .
 apocrine carcinoma ( ac ) is a rare tumor with heterogeneous presentation . 
 the disease has a highly morbid course and little is known about it . 
 we present an otherwise healthy , 62-year - old asian male who originally presented with chronic swelling of his left eyelid associated with excessive tears and diminished vision was diagnosed with ac . 
 ac is often challenging to diagnose , yet it is critical to do so as early diagnosis and treatment can maximize patient survival . 
Introduction
Case Report
Discussion
Conclusions