Eccrine Porocarcinoma Associated with an Eccrine Poroma

Manimaran D

ISSN 0972-5997

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Mangalore, India

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OJHAS Vol. 13, Issue 2: (Apr-Jun 2014)

Case Report
Eccrine Porocarcinoma Associated with an Eccrine Poroma.

Authors
Manimaran D, Associate Professor,
Thulasi Raman R, Assistant Professor,
Hemanathan G, Postgraduate Student,
Ishwarya S, Postgraduate Student,
Anuradha S, Professor & Head,
Department of Pathology, Shri Sathya Sai Medical College and Research Institute, Nellikuppam, Chengalpet Taluk, Kancheepuram District, Tamil Nadu, India.

Address for Correspondence
Dr. Manimaran D, Associate Professor,
Department of Pathology,
Shri Sathya Sai Medical College and Research Institute,
Tiruporur-Guduvanchery Main Road,
Ammapettai, Nellikuppam-603108,
Chengalpet Taluk, Kancheepuram District,
Tamil Nadu, India.
E-mail: manimaran.anu@gmail.com

Citation
Manimaran D, Thulasi RR, Hemanathan G, Ishwarya S, Anuradha S. Eccrine Porocarcinoma Associated with an Eccrine Poroma. Online J Health Allied Scs. 2014;13(2):5. Available at URL: http://www.ojhas.org/issue50/2014-2-5.html

Open Access Archives
http://cogprints.org/view/subjects/OJHAS.html
http://openmed.nic.in/view/subjects/ojhas.html

Submitted: May 24, 2014; Accepted: Jul 16, 2014; Published: Aug 30, 2014

This work is licensed under a Creative Commons Attribution-No Derivative Works 2.5 India License

This Article

Abstract: Eccrine porocarcinoma is a rare carcinoma arising from the intraepidermal duct of eccrine sweat glands and constitute about 0.005% of epithelial cutaneous neoplasms. Eccrine porocarcinoma is commonly seen in lower extremities, followed by head, scalp, upper extremities, trunk and abdomen. It can arise denovo or from pre-existing eccrine poroma in 18% of cases. We present a 45 years old female patient who presented with painful hard warty growth of size 3x2 cm with ulceration and an adjacent nodule measuring 1x1 cm in the lateral aspect of dorsum of left foot for the past 3 years with rapid increase in size for 3 months duration. Microscopically larger and smaller nodules showed features of eccrine porocarcinoma and eccrine poroma respectively. Immunohistochemistry for pancytokeratin showed strong membrane positivity, Ki-67 showed 40% nuclear positivity and CD 34 negative. The lesion was diagnosed microscopically as eccrine porocarcinoma associated with pre-existing eccrine poroma.
Key Words: Eccrine porocarcinoma, Sweat gland carcinoma, Malignant eccrine poroma.

Introduction:

Eccrine porocarcinoma (EPC) is a rare adnexal carcinoma arising from the intraepidermal duct (acrosyringium) of eccrine sweat glands. The first case was reported in 1963 by Pinkus and Mehregan.[1] They called it ‘epidermotropic eccrine carcinoma’. In 1969 Mishma and Morioka introduced the term ‘eccrine porocarcinoma’. EPC constitute about 0.005% of epithelial cutaneous neoplasms and commonly seen in lower extremities, followed by head, scalp, upper extremities, trunk and abdomen.[2] It can arise denovo or from pre-existing eccrine poroma. We present a case of eccrine porocarcinoma associated with pre-existing eccrine poroma of long duration with clinical history of pain and sudden increase in size.

Case Report:

A 45 years old female patient presented with painful hard warty growth of size 3x2 cm with ulceration and an adjacent nodule measuring 1x1 cm in the lateral aspect of dorsum of left foot for the past 3 years. The lesion presented as a small nodule and showed rapid growth for the past 3 months to attain the present size. The lesion was excised and sent for histopathological examination. Grossly the specimen showed wedge of skin with two nodules larger measuring 3x2 cm with an ulcer 1x1cm and an adjacent smaller nodule measuring 1x1cm (Fig – 1). Cut section showed gray white ill-defined nodular lesion in the dermis measuring 3x3 cm with areas of haemorrhage and an adjacent nodule measuring 1x1 cm (Fig – 2).


Fig 1: Specimen shows wedge of skin with two nodules larger measuring 3x2 cm with an ulcer 1x1cm and an adjacent smaller nodule measuring 1x1cm.	Fig 2: Cut section showed gray white ill-defined nodular lesion in the dermis measuring 3x3 cm with areas of haemorrhage and an adjacent gray white nodule measuring 1x1 cm.

Microscopically the larger lesion showed ulcerated squamous epithelium with underlying malignant cells arranged in sheets and clusters with marked nuclear pleomorphism, prominent nucleoli, areas of necrosis and invasion in to the deeper tissue. At places ductal structures seen with accumulation of pink secretory material which was periodic acid Schiff stain positive (Fig-3,4). Section from the smaller nodule showed features of eccrine poroma composed of epidermis with underlying dermis showing broad, anastomosing bands of small epithelial cells in continuity with epidermis and composed of intercellular bridges and round basophilic nucleus (Fig-5). Immunohistochemistry for pan cytokeratin and Ki-67 was done in the larger lesion. CD 34 was also done to rule out vascular lesion because of increased vascularity noted in the lesion. Pancytokeratin staining showed strong membrane positivity (Fig-6). Ki-67 staining was 40% positive and it indicated the high proliferation rate of the tumor (Fig-7). CD 34 stained the endothelial cells of blood vessels but not the tumor cells (Fig-8). Surgical margins are free from tumor invasion. Histopathological diagnosis was porocarcinoma probably arising from pre-existing eccrine poroma. No metastatic deposits were detected in the patient and follow up till date is uneventful.


Fig 3: Show malignant cells arranged in sheets and clusters separated by fibrous stroma and few ducts with secretory material (H&E, 10x).	Fig 4: Show malignant cells with marked nuclear pleomorphism, prominent nucleoli and duct formation with secretory material (H&E, 40x). Inset show Periodic acid Schiff positive material in the duct lumen (PAS stain, 40x)

Fig 5: Section from the smaller nodule showed features of eccrine poroma composed of broad, anastomosing bands of small epithelial cells in continuity with epidermis (H&E,4x)	Fig 6: Immunohistochemistry marker Pancytokeratin showed strong membrane positivity (4x)


Fig 7: Ki-67 immunohistochemistry marker showed 40% positivity (10x).	Fig 8: Immunohistochemistry marker CD 34 stained only endothelial cells and not tumor cells (4x)

Discussion

Eccrine unit consists of coiled tubes at both ends connected by straight tube which runs in the dermis. The deep coiled part is the secretory unit, the intraepidermal coiled part is known as acrosyringium and straight part is called syrinx.[3] Eccrine poroma and eccrine porocarcinoma arise from acrosyringium. Eccrine porocarcinoma (EPC) is usually seen in sixth to eight decades of life with female predominance.[1] In around 50% of cases the site was lower limbs and 40% out of these cases occurred below knee. Other sites are head and neck, scalp, upper extremities, trunk and abdomen.[2] Scalp lesion can sometimes present with intracranial extension.[4] EPC usually metastasize through lymphatics to the regional lymph nodes.[5] There can be multiple cutaneous metastases or visceral metastases to lung, retroperitoneum, bones, breast, liver, mediastinum, urinary bladder, and ovary.[1] EPC arise denovo from the intraepidermal portion of eccrine duct or from pre-existing eccrine poroma. Robson et al reported occurrence of EPC in 18% of eccrine poroma.[6] This was supported by long clinical history of a nodular lesion with sudden increase in size. Symptoms like pain, itching and bleeding are in favour of malignant transformation.[6] Malignant change may be induced by immunosuppression or ionizing radiation.[4] Association with lymphocytic dysfunction like multiple myeloma and chronic lymphocytic leukemia had been noted but the mechanism is not clearly understood.[4] Table I shows characteristics of EPC in literature in comparison with our case.

Table 1: Summary of Eccrine porocarcinoma cases in literature.
Author	Age / sex	Duration	Site	Size (cms)	Metastasis	Association with eccrine poroma
Choi CM et al[1]	44/M	7 months	Right scrotum	7x6	Lymphnodes, Lungs, Adrenal, Esophagus	No
Chang O et al[2]	42/M	6 years	Right lower limb	3x2x0.6	Absent	Yes
Moussallem CD et al[5]	77/M	4 months	Right 1st toe		Absent	No
Gerber PA et al[4] (5 cases)	1)64/F	4 years	Scalp	3	Absent	No
	2)77/M	2 months	Scalp	2	Absent	No
	3)80/M	3 years	Scalp	3	Absent	No
	4)78/M	1 year	Scalp	4	Absent	No
	5)79/M	1 year	Left retroauricular	3	Absent	No
Latifi H et al[7]	72/M	30 years	Left cheek	5x5	Absent	No
Marone U et al[8]	42/M	10 months	Left arm	2	Left Axillary lymphnodes	No
Present case	45/F	3 years	Dorsum of left foot	3x2	Absent	Yes

Clinically EPC occurs as a solitary, slow growing, pink to red nodule with smooth or ulcerated surface. Differential diagnosis includes basal cell carcinoma, squamous cell carcinoma, amelanotic melanoma, metastatic carcinomas and other malignant adnexal tumors.[7] Local recurrence and lymphnode metastasis occur in 20% cases and distant metastasis occur in 10% cases.[8]

Treatment is wide surgical excision for localised tumors, regional lymphadenectomy for nodal deposits, adjuvant chemotherapy and radiotherapy for wide spread tumors. Cure rate after wide surgical excision for tumors without metastasis is 80% but prognosis is poor for patients with lymphnode deposits or distant metastasis.[2,6,9] In our case there was no nodal or distal metastasis and follow up till date is uneventful.

Conclusion

As in our case, EPC associated with eccrine poroma typically will have a long clinical history with sudden increase in size or may present with bleeding, itching and pain. EPC should be considered in the differential diagnosis of skin tumours in the elderly. Early surgical intervention is curative and is essential to avoid transformation of benign lesions to malignancy.

References

Choi CM, Cho HR, Lew BL, Sim WY. Eccrine porocarcinoma presenting with unusual clinical manifestation: A case report and review of the literature. Ann Dermatol. Sep 2011 ;23 suppl 1: S79-83.
Chang O, Elnawawi A, Rimpel B, Asarian A, Chaudhry N. Eccrine porocarcinoma of the lower extremity: A case report and review of literature. World J Surg Oncol. 2011Aug 22;9:94.
Sigal R, Tordeur M, Avril MF, Spatz A, Vanel D. Eccrine porocarcinoma with intracerebral extension. Eur Radiol 1997;7(4):573-5.
Gerber PA, Schulte KW, Ruzicka T, Bruch-Gerharz D. Eccrine porocarcinoma of the head. All important differential diagnosis in the elderly patient. Dermatology. 2008;216(3):229-33.
Moussallem CD, Abihatem NE, El-khoury ZN.Malignant porocarcinoma of the nail fold: A tricky diagnosis. Dermatol Online J 2008 Aug 15;14(8):10.
Zhang L, Ge S, Fan X. A brief review of different types of sweat-gland carcinomas in the eyelid and orbit. Onco Targets Ther. 2013 Apr 9;6:331-40.
Latifi H, Mikaili P, Rozbehani NA, Taherian S, Latifi K. Malignant eccrine poroma (eccrine porocarcinoma): report of a clinical case. Comp Clin Pathol 2012;21:1131-33.
Marone U, Caraco C, Anniciello AM, Di Monta G, Chiofalo MG, Cecilia ML, Mozzillo N. Metastatic eccrine porocarcinoma: report of a case and review of the literature. World J Surg Oncol. 2011 Mar 16;9:32.
Rana RE, Verma SS, Puri VA, Baliarsing AS. Sweat gland tumor (eccrine porocarcinoma) of scalp: A rare tumor. Indian Journal of Plastic Surgery. 2005;38(1):51-53.