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Year : 2017  |  Volume : 13  |  Issue : 6  |  Page : 1038-1041

Multinodular fibroepithelioma of Pinkus with an isolated focus of evolving basal cell carcinoma in chronic lymphedematous lower limb

1 Department of Pathology, G.B. Pant Institute of Medical Education and Research, Jawaharlal Nehru Marg, New Delhi, India
2 Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Bhopal, Madhya Pradesh, India
3 Department of Dermatology, All India Institute of Medical Sciences, Bhopal, Madhya Pradesh, India

Date of Web Publication13-Dec-2017

Correspondence Address:
Dr. Kaushik Majumdar
Department of Pathology, G.B. Pant Institute of Medical Education and Research, (Maulana Azad Medical College), Jawaharlal Nehru Marg, Academic Block, Room No. 323, New Delhi - 110 002
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.220454

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 > Abstract 

Fibroepithelioma of Pinkus (FEP) is a rare, indolent variant of basal cell carcinoma (BCC) with a unique anastomosing type of epidermal hyperplasia. Certain benign and neoplastic conditions including BCC are known to develop in a setting of chronic lymphedema. We report for the 1st time the histological spectrum of evolution of a large exophytic multinodular FEP, bearing a single initiating focus of BCC, in preexisting chronic lymphedema of the lower limb. The lesion possibly evolved from long-standing local immune suppression, and clinically resembled a high-grade carcinoma. Oozing lymphatic fluid may interfere with the success of skin grafting following wide local excision in a lymphedematous limb, thus offering a challenging scenario from the management point of view.

Keywords: Basal cell carcinoma, fibroepithelioma of Pinkus, leg, lymphedema

How to cite this article:
Majumdar K, Khurana U, Asati D, Goel G, Joshi D, Kapoor N. Multinodular fibroepithelioma of Pinkus with an isolated focus of evolving basal cell carcinoma in chronic lymphedematous lower limb. J Can Res Ther 2017;13:1038-41

How to cite this URL:
Majumdar K, Khurana U, Asati D, Goel G, Joshi D, Kapoor N. Multinodular fibroepithelioma of Pinkus with an isolated focus of evolving basal cell carcinoma in chronic lymphedematous lower limb. J Can Res Ther [serial online] 2017 [cited 2020 Mar 31];13:1038-41. Available from: http://www.cancerjournal.net/text.asp?2017/13/6/1038/220454

 > Introduction Top

Chronic lymphedema precipitates an immunocompromised milieu for the development of certain benign or potentially malignant skin lesions.[1],[2],[3],[4],[5] We report for the 1st time an unusual case of fibroepithelioma of Pinkus (FEP) presenting as an exophytic ulcero-nodular growth, in preexisting chronic lymphedema lower limb of 6 years duration. The histological spectrum of the evolution of the lesion, differential diagnoses and management concerns have been discussed, and a brief review of the available literature has been attempted.

 > Case Report Top

A 65-year-old man with tanned complexion presented with lymphedema of the lower extremities (right more than left) of 6 years duration, along with a polypoid ulcero-nodular fleshy pinkish growth developing over a span of 6 months, in the anterior aspect of right lower limb [Figure 1]. The growth had a broad base, covering an area of 6.5 cm × 5.5 cm, with multiple superficial bleeding points and blackish discoloration of the adjoining skin. He had no history of prior surgery, pelvic or lower extremity infections or immunosuppressive medications. There was also no history of excessive sun exposure as he worked indoors during his profession. The cardiopulmonary profile was normal, and blood pressure measured on multiple occasions was within normal range. A physical examination and imaging did not reveal any inguinal or intra-abdominal lymphadenopathy. The complete hemogram, serum albumin, liver and renal function tests were all within the normal range, and the secondary causes of lymphedema including lymphatic filariasis have been meticulously ruled out. He was also nonreactive for HIV, hepatitis B virus or hepatitis C virus.
Figure 1: Marked lymphedema right leg showing a multinodular polypoid pinkish growth, with blackish discoloration of the surrounding skin and oozing lymphatic fluid

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Earlier, a 99m Tc sulfur colloid lymphoscintigraphy scan revealed radiotrace accumulation at injection sites in all four limbs, with progressive transit through lymphatics. Visualization of conspicuous draining lymph nodes, including bilateral inguinal and pelvic nodes, was possible. However, dermal backflow of the tracer was noted in the right lower limb, suggesting partial lymphatic obstruction in right lower limb and patent lymphatics in all other limbs.

The patient underwent biopsies from different representative sites with a clinical possibility of high-grade carcinoma. Sections examined showed an elevated polypoid lesion with surface ulceration and fibrinous exudate. The epidermis showed hyperkeratosis, papillomatosis, and areas of pseudoepitheliomatous hyperplasia. Multiple thin interlacing epithelial strands were seen protruding down from the epidermis into the underlying dermis, embedded in a fibrovascular stroma [Figure 2]. These epithelial cords were 2–3 cell layers thick and showed focal peripheral palisading with mild nuclear atypia. Immunohistochemistry for CD31 highlighted the vascular endothelium in the fibroconnective stroma [Figure 3]. A diagnosis of FEP was offered, after ruling out the differential diagnoses of a reticulated variant of seborrhoeic keratosis and eccrine syringofibroadenoma.
Figure 2: (a and b) Photomicrographs in scanner view showing epidermis with hyperplasia, papillomatosis, focal ulceration, and cornification (H and E, ×40); (c) multiple thin anastomosing cords of basaloid cells extending downward from the epidermis, embedded in an edematous fibrovascular stroma (H and E, ×40); (d) encircled area of “c” in higher magnification (H and E, ×100)

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Figure 3: Immunohistochemistry for CD31 highlighting the vascular endothelium (×200)

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One isolated tiny focus of invading islands with peripheral palisading, nuclear hyperchromasia, and retraction artifact was observed, representing the grim onset of a basal cell carcinoma (BCC) [Figure 4]. Examination of rest of the skin did not reveal any other evidence of neoplasia or lesions suggestive of actinic keratosis. The patient has been using pneumatic device for both lower limbs, following which the lymphedema has reduced. The clinical team is expecting a regression of the extent of the lesion with further improvement of lymphedema, and wide local excision with skin grafting may be planned subsequently.
Figure 4: (a) Isolated invading islands showing peripheral palisading and nuclear atypia – heralding the onset of a basal cell carcinoma (H and E, ×40); (b) encircled area of “a” in higher magnification: “Cancer initiates its pincer grasp”; retraction artifact (arrow) (H and E, ×200)

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 > Discussion Top

FEP has been presently considered as a rare low-grade BCC variant with a unique type of epidermal hyperplasia.[6] The lesion occasionally may evolve into or merge with a full blown nodular BCC.[7]

Lymphedema may be primary or secondary the latter being more common. Primary lymphedema may be due to congenital or acquired obstruction or obliteration of the lymphatics, usually due to unknown etiology. Secondary lymphedema may be due to malignancies or associated treatments, surgical procedures, radiation therapy, autoimmune or infective conditions.[8] This patient might be having acquired primary lymphedema due to the obliteration of distal lymphatics.[8]

Abnormalities appreciated in lymphoscintigraphy of lymphedema include dermal backflow, absent or delayed transport of tracer, cross-over filling with retrograde backflow, and absent or delayed visualization of the lymph nodes.[8] In this patient, dermal backflow of the tracer was noted in the right lower limb, suggesting partial lymphatic obstruction.

Stasis impairs circulation of immune reactive lymphocytes, resulting in a loco-regional immunodeficient state and susceptibility to carcinogenesis. Impairment of lymphatic drainage may predispose to skin lesions such as stasis dermatitis, cellulitis, or erysipelas.[4] The neoplasms arising from the tissue compromised by chronic lymphedema include Stewart–Treves angiosarcoma in posttherapy breast cancer, basal, and squamous cell carcinomas, Kaposi's sarcoma, lymphoma, and melanoma.[1],[2],[3],[4],[5] On the other aspect, the risk factors for BCC include ultraviolet radiation, genetic predisposition, fair skin, old age, and immunosuppression.[5]

FEP occurs in the middle-aged to elderly, commonly in the lumbosacral, inguinal region, or inferior extremities.[9] It manifests clinically as a solitary or multiple, flesh-colored well-demarcated polypoid lesion, with surface ulceration and invasion into underlying tissues.[9] Dermoscopic findings useful in the diagnosis are fine arborizing vessels, peripheral dotted vessels, and white streaks.[9]

Histologically FEP represents an anastomosing type of epidermal hyperplasia.[6] The coexpression of cytokeratin 20, androgen receptor, and PHLDA1 may serve as an immunohistochemical supplement in diagnosis.[6],[10] In this patient, the local immunosuppressive milieu due to chronic lymphedema has provided the nidus for epidermal hyperplasia, which progressed to extensive ulcero-nodular FEP. In addition, the single tiny focus of invading islands with peripheral palisading and nuclear hyperchromasia has probably represented the onset of a BCC. Hence, a continuum of histological changes from epidermal hyperplasia through FEP and finally to the initiation of BCC has been observed in this long-standing lymphedematous lower limb.

The histological differential diagnoses considered were an adenoid or reticulated variant of seborrheic keratosis, eccrine syringofibroadenoma, a tumor of follicular infundibulum and BCC. The clinical appearance did not resemble a seborrheic keratosis. In addition, hyperpigmentation of basaloid cells and presence of horn cysts were not observed. Eccrine syringofibroadenoma is a plaque like lesion extending several centimeters in diameter and is composed of similar interweaving cords; however, here, the cords are much slender and are not basaloid, often with lumina formation and clear cell change. The tumor of follicular infundibulum usually presents as a keratotic papule and shows a horizontal plate-like growth of epithelial cells with multiple connections to the lower border of the epidermis. Horn cyst formation and cytoplasmic clearing are conspicuous features.

Lymphedema may be treated conservatively by means of compression therapy, such as pneumatic pumps and compressive garments. Volume reducing surgery and lymphatic microsurgery are also performed rarely.[8] For FEP in this patient, adequate resection with full-thickness skin grafting should be recommended.[5] However, healing and survival of the skin graft may be unpredictable due to impaired lymphatic drainage.

To conclude, FEP is a rare, indolent variant of BCC with unique growth pattern and no metastatic potential. An emerging spectrum from epidermal hyperplasia through FEP, to the initiation of BCC has been described for the 1st time in the chronic lymphedematous lower limb. The extensive ulcero-nodular appearance in this case clinically resembled a high-grade carcinoma. The outcome of wide local excision and successful skin grafting in a lymphedematous limb may be unpredictable.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Ruocco V, Schwartz RA, Ruocco E. Lymphedema: An immunologically vulnerable site for development of neoplasms. J Am Acad Dermatol 2002;47:124-7.  Back to cited text no. 1
Peyron N, Dandurand M, Guillot B. Malignant tumors as complications of lymphedema. J Mal Vasc 1993;18:293-8.  Back to cited text no. 2
Benson PM, Pessoa CM, Lupton GP, Winton GB. Basal cell carcinomas arising in chronic lymphedema. J Dermatol Surg Oncol 1988;14:781-3.  Back to cited text no. 3
Lotem M, Tamir G, Loven D, David M, Hauben D. Multiple basal cell carcinomas of the leg after recurrent erysipelas and chronic lymphedema. J Am Acad Dermatol 1994;31 (5 Pt 1):812-3.  Back to cited text no. 4
Ueno T, Futagami A, Mitsuishi T, Niimi Y, Shimoda T, Kawana S. Basal cell carcinoma arising on a chronic lymphedematous leg. J Dermatol 2009;36:646-8.  Back to cited text no. 5
Sellheyer K, Nelson P, Kutzner H. Fibroepithelioma of Pinkus is a true basal cell carcinoma developing in association with a newly identified tumour-specific type of epidermal hyperplasia. Br J Dermatol 2012;166:88-97.  Back to cited text no. 6
Misago N, Suzuki Y, Miura Y, Narisawa Y. Giant polypoid basal cell carcinoma with features of fibroepithelioma of Pinkus and extensive cornification. Eur J Dermatol 2004;14:272-5.  Back to cited text no. 7
Szuba A, Rockson SG. Lymphedema: Classification, diagnosis and therapy. Vasc Med 1998;3:145-56.  Back to cited text no. 8
Zalaudek I, Ferrara G, Broganelli P, Moscarella E, Mordente I, Giacomel J, et al. Dermoscopy patterns of fibroepithelioma of Pinkus. Arch Dermatol 2006;142:1318-22.  Back to cited text no. 9
Katona TM, Ravis SM, Perkins SM, Moores WB, Billings SD. Expression of androgen receptor by fibroepithelioma of Pinkus: Evidence supporting classification as a basal cell carcinoma variant? Am J Dermatopathol 2007;29:7-12.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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