|Year : 2018 | Volume
| Issue : 4 | Page : 156-158
Primary mucinous eccrine adenocarcinoma of anterior chest wall
M Ramula, M Mohan Raj, R Ram Prasath
Department of General Surgery, Karpaga Vinayaga Institute of Medical Science, Kanchipuram, Tamil Nadu, India
|Date of Web Publication||13-Dec-2018|
Dr. M Ramula
Department of General Surgery, Karpaga Vinayaga Institute of Medical Science, Madurantakam, Kanchipuram, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Mucinous eccrine carcinoma is a very rare cutaneous malignancy, rarely documented in literature (0.05%.) It is commonly found in lower extremities. It is a tumor of old age. Diagnosis is mainly by histopathological report, as clinical diagnosis is very difficult. A 60-year-old female residing nearby village presented with lump left breast of 6 months. A solitary lump left breast was present over nipple areola with no regional lymph nodes. Mucinous eccrine tumors are common in the 6th decade with male: female ratio 2:1. They present as nodular cutaneous swelling with no distinct clinical features, making it difficult to diagnose preoperatively. They metastasize to regional lymph nodes, lungs, liver, and bones. Five-year survival rate is 59% without metastasis. It comes down by 9% with lymph node involvement. Surgery is the principal mode of treatment; Histopathological examination confirms the diagnosis and since the cells are rich in glycogen they stain with PAS. Difficult to distinguish except by Hisochemistry from adnexal tumors. Immunohistochemistry aids in confirming the diagnosis. Primary mucinous adenoma with reported incidence of 0.05% is one of the rarest tumors. Immunohistochemistry complement in confirming the diagnosis. Wide excision is the treatment of choice. Close follow-up is essential.
Keywords: Adnexal tumor, breast, mucinous eccrine
|How to cite this article:|
Ramula M, Raj M M, Prasath R R. Primary mucinous eccrine adenocarcinoma of anterior chest wall. Saudi Surg J 2018;6:156-8
| Introduction|| |
Primary mutinous adenocarcinoma is a rare adnexal neoplasm. The common site of occurrence is head and neck. In this article, we report the rare occurrence of this adnexal tumor in nipple-areola complex, which has been reported in only 0.05% of cases. Metaststic tumours from primary such as Breast and GI tract are difficult to differentiate from primary adnexal tumours. Diagnosis of this tumor is very difficult because of the paucity of literature. primary mucinous carcinoma more common in 5th to 7th decade. male to female ratio is 2 : 1. Surgery is the treatment of choice.
This a rare case of adnexal tumour presented as right breast lump in 60 year old female. This is reported for its rarity.
| Case Report|| |
A 60-year-old female patient, habitant of nearby village, presented herself with a lump left breast of 6-month duration. On local examination revealed a single lobulated lump of size 5x3 cm present over left nipple and areola, It was non tender, mobile, Fixed to skin, not fixed to underlying structures. Clinically no palpable regional lymph nodes. The other side breast and abdominal examination was normal. A provisional diagnosis of carcinoma breast was made.
Routine investigations were within normal limits.
Smear shows high cellularity with abundant clusters of acinar figures, cords in a mucinous background with eosinophilic protinecious substance.
Mild pleomorphic nuclei showing occasional intranuclear Occasional intranueclear inclusions and plewomorphic nueclei also noted.
Fine-needle aspiration cytology (FNAC) was done on the day of admission, which suggested the differential diagnosis of:
- Metastatic carcinomatous deposits
- Malignant skin adnexal tumor which suggested wide excision and HPE to confirm the diagnosis.
No other primary could be detecteed by clinical, biochemical and radiological investigation.
Based on cytological report, and preclincal work up, decided to proceed Wide local excison. Informed consent from the patient and the proposed management plan discussed before surgery. Under general anesthesia, a wide excision of lump with the surrounding healthy skin was done. Specimen was sent for histopathological examination.
Histopathology report: (May 5, 2018) Skin lined by stratified squamous epithelium, with focal ulceration and subepithelium showing a malignant neoplasm arranged in solid nests, cord and tubular pattern separated by fibrous septa, in a abundant mucinous background. Individual cells are polyhedral with mild pleomorphic nuclei, moderate eosinophilic to clear vacuolated cytoplasm some of them exhibiting signet-ring morphology. Resected margins free of tumor impression.
Patient had uneventful post operative period hence discharged after a week, coming for regular follow up after review at near by cancer institute.
| Discussion|| |
Primary eccrine mucinous adeno carcinoma is a rare cutaneous malignant tumor, indistinguishable from metastatic tumors of the breast. They do not have characteristic clinical features which makes diagnosis difficult, so eccrine tumors are rarely diagnosed clinically. They normally present as nodules more commonly seen in elderly. Average age being in the 6th decade.
Because of varieid clinical presentation these tumours are difficult to make a definitive preoperative diagnosis.
The site of metastasis includes lung liver and bones.
Many studies have shown that immunohistocemistry does not distinguish cutaneous eccrine tumors from metastases of the breast. John A Papalas is his article reported that both primary adnexal tumours and breast tumours are CK 7 positve and negative for CK 20. In metastatic breast tumours expression P53 AND CK5/6 are negattive.
Clinically eccrine tumor is considered as differential diagnosis for Paget's disease. HPE of the mucinous eccrine tumor is similar to the counterpart in the breast. Eccrine tumors have the potential for local traction and regional metastasis. Prognostic features include size, lymph node involvement and distant metastasis.
Five year survival reported to be 59% in many articles if there is no proven nodal invovlement. Survival rate comes down to only 9%, if there is a nodal invovlement. Nutrition of the cells afftected by mucin secretion prime reason for slow growth and metastasis.
| Conclusion|| |
Primary cutaneous mucinous eccrine adino-carcinoma in a rare site like breast is difficult to diagnose. It is often diagnosed by FNAC and confirmed by HPE. These tumors cause local destruction and distant metastasis. Treatment is usually wide excision. In this case, no lymph node metastasis was present, but the patient must be followed up regularly for recurrence and lymph node metastasis. Treatment of choice is wide local excision and close follow up. Lymph node clearance indicated in case of lymphnode involvement.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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