gms | German Medical Science

GMS German Plastic, Reconstructive and Aesthetic Surgery – Burn and Hand Surgery

Deutsche Gesellschaft der Plastischen, Rekonstruktiven und Ästhetischen Chirurgen (DGPRÄC)
Deutsche Gesellschaft für Verbrennungsmedizin (DGV)

ISSN 2193-7052

Synchronous atypical fibroxanthoma and Bowen’s disease of the head and neck in an otherwise normal patient – a case report and review of literature

Simultanes Auftreten eines atypischen Fibroxanthoms und Plattenepithelkarzinoms im Kopf-Hals-Bereich – Fallbericht und Literaturübersicht

Case Report

  • corresponding author Ahmad Eweida - Department of Plastic and Hand Surgery, University of Erlangen Medical Centre, Erlangen, Germany; Department of Head and Neck Surgery, University of Alexandria, Egypt
  • author Elias Polykandriotis - Department of Plastic and Hand Surgery, Sana Klinikum Hof, Germany
  • author Marweh Schmitz - Department of Plastic and Hand Surgery, University of Erlangen Medical Centre, Erlangen, Germany
  • author Stephan Schwarz - Institute of Pathology, Friedrich Alexander University Erlangen, Erlangen, Germany
  • author Juergen Bauerschmitz - Department of Dermatology, University of Erlangen Medical Centre, Erlangen, Germany
  • author Adrian Dragu - Department of Plastic and Hand Surgery, University of Erlangen Medical Centre, Erlangen, Germany
  • author Raymund E. Horch - Department of Plastic and Hand Surgery, University of Erlangen Medical Centre, Erlangen, Germany

GMS Ger Plast Reconstr Aesthet Surg 2012;2:Doc04

DOI: 10.3205/gpras000006, URN: urn:nbn:de:0183-gpras0000066

Veröffentlicht: 15. März 2012

© 2012 Eweida et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.de). Er darf vervielfältigt, verbreitet und öffentlich zugänglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.


Abstract

Although both atypical fibroxanthoma and squamous cell carcinoma arise on top of sun-damaged skin of the elderly, there is no evidence in literature reporting a synchronous presentation of primary lesions of both malignancies in the head and neck regions.

We report a case of synchronous atypical fibroxanthoma and squamous cell carcinoma in situ (Bowen’s disease) of the head and neck in an otherwise normal old Caucasian male patient. We reviewed the literature for cases of head and neck atypical fibroxanthoma in association with other skin malignancies with an overview over the risk factors and modalities of treatment. We would like to raise the awareness for the concept of multiple synchronous primary malignant lesions and the importance to anticipate and differentiate between different pathologies in order to provide adequate investigations and treatment for the patient.

Keywords: atypical fibroxanthoma, squamous cell carcinoma, synchronous cancers

Zusammenfassung

Obwohl sowohl das atypische Fibroxanthom der Haut als auch das Plattenepithelkarzinom bei sonnengeschädigter Haut älterer Menschen gehäuft vorkommen, gibt es in der Literatur keine Beschreibung eines simultanen Auftretens beider Malignome als Primärläsionen im Kopf-Hals-Bereich. Aus diesem Grund möchten wir über einen Fall berichten, bei dem sowohl ein atypisches Fibroxanthom als auch ein Morbus Bowen (als in situ Variante des Plattenepithelkarzinoms) im Kopf-Hals-Bereich eines sonst gesunden 74-jährigen männlichen Patienten aufgetreten ist. Auf Grundlage der bestehenden Publikationen von Fällen mit atypischen Fibroxanthomen in Assoziation mit anderen Hautmalignomen, verglichen wir diese mit dem vorliegenden Fall, insbesondere in Hinblick auf die Risikofaktoren und das empfohlene Behandlungsregime. Ein simultanes Auftreten von mehreren primären Hauttumoren verschiedener Entität ist in einigen Fällen möglich und sollte daher insbesondere bei älteren Menschen deutlich häufiger in die Beurteilung und Diagnosestellung mit eingeschlossen werden. Ein Hauptaugenmerk sollte hierbei insbesondere auf die Differenzierung dieser verschiedenartigen Pathologien zu liegen kommen, um so eine inadäquate Therapie zu vermeiden.


Introduction

The risk factors that contribute to the development of the non-melanoma skin cancer (NMSC) include ethnicity, age, gender, chronic exposure to chemical and physical mutagens, besides genetic factors [1]. Excessive exposure to ultraviolet (UV) radiation, especially type B (UVB), has been associated with a high risk of developing skin cancers, namely squamous cell carcinoma (SCC) and basal cell carcinoma (BCC). The DNA damage due to UVB results in actinic keratoses (AK), solar lentigo, and Dermatoheliosis (i.e., “photoaging”) [2]. A small percentage of squamous cell carcinomas, however, do not follow these pathological changes stimulated by UV radiation exposure. In such cases, skin cancer may arise in skin wounds, and immunosuppressed persons may have higher incidence [3]. By far, the most frequent types of epithelial skin cancers are BCC and SCC [4], [5]. Atypical fibroxanthoma (AFX) is relatively uncommon, and was first was first defined by Helwig in 1961 to describe some cutaneous tumors with marked cellular pleomorphism but benign clinical course [6]. AFX is considered now a malignant fibrohistiocytic neoplasm that most commonly arises on sun damaged skin of elderly individuals [7].

Patients with head and neck skin cancers frequently develop multiple skin cancers. According to the timing of presentation, these multiple lesions could be classified as synchronous or metachronous lesions. Multiple synchronous primary lesions (SEER Program Coding and Staging Manual 2004, Revision 1) are those primary lesions with different pathology, occurring within 2 months of each other. Being aware of this concept is important in the clinical practice in order to be able to treat and investigate the patient adequately according to the presented lesions. Bearing in mind that a patient may have a totally different synchronous primary lesion can influence further investigations of the patient and can avoid inappropriate management procedures that may be considered undertreatment or sometimes an overtreatment.

We present here a case of synchronous AFX and SCC in situ occurring in the head and neck region.


Case report

We report a case of synchronous AFX and SCC in situ (Bowen’s disease) in the skin of the Head and neck region. The patient is a 74 year old Caucasian male, working as field engineer, with no history of irradiation or immunosuppressive therapy. The patient presented to the out patient clinic with multiple scalp and nasal lesions (Figure 1 [Fig. 1]). Immunohistochemically confirmed biopsies from the scalp reported atypical fibroxanthoma (positive for vementin, negative for S100, CD31, and cytokeratin) (Figure 2 [Fig. 2]). Two other suspected lesions from the left side of the nose and the right side of the scalp were biopsied and revealed squamous cell carcinoma in situ (Bowen’s disease). The lesions were reported to be surrounded with atrophic multifocal actinic keratosis, as well as massive solar elastosis. Definitive treatment of the patient was done by wide surgical excision of the lesions with appropriate safety margins (R0 resection) (Figure 3 [Fig. 3]; left). A scalp lesion was closed with split thickness skin graft, and the nasal defect was treated with a delayed glabellar local flap (Figure 3 [Fig. 3]; right). The postoperative period passed uneventful and the patient was regularly followed up for eventual appearance of other lesions, recurrence or metastasis (Figure 4 [Fig. 4]). Five months later, the patient developed another small (1 cm) SCC lesion at the back of the scalp that was surgically removed (R0) and the defect was covered using a full thickness skin graft. The patient has undergone an esthetic correction operation for the nose aperture 6 months later. No recurrence or metastasis was diagnosed through a 3-year follow up period. The patient was advised to avoid direct sun light exposure, was encouraged to make regular follow up visits, and to promptly seek medical advice on appearance of any suspicious new skin lesions.


Discussion

In our case, the AFX stained negative for cytokeratin. Mirza and Weedon previously studied 89 cases of AFX and found cytokeratin expression in only one of the lesions that displayed dual features of AFX and squamous cell carcinoma. Based on these data, they concluded that it appears unlikely that AFX is a variant of squamous cell carcinoma [8]. This confirms the unique identity of each of the tumours and thus defines both tumours occurring in the same patient as multiple synchronous primary tumors.

In our case the lesions were reported to be surrounded with atrophic multifocal actinic keratosis, as well as massive solar elastosis. Actinic keratosis is defined as a precursor to SCC, and studies have shown that most cases of SCC arise from AK lesions [9], [10], [11].

To the best of our knowledge, this is the first report of synchronous AFX and SCC (CIS) occurring in the region of the head and neck in a non- XP (Xeroderma Pigmentosa) patient occurring on the native skin of the patient (not on burn scars or grafted skin). Youssef et al. previously reported about a case of synchronous AFX and basisquamous cell carcinoma in the face. The 6 year-old- girl however was suffering from XP [12]. A 66-year-old male patient was reported with several squamous cell carcinomas and a myxoid atypical fibroxanthoma of the skin. The tumours developed in burn scars that the patient had had for more than 50 years and that, in part, undergone actinic elastosis [13]. A reported case of atypical fibroxanthoma, lentigo malignant melanoma and squamous cell carcinoma developed in the site of a thermal burn that had previously been treated with multiple skin grafts. The SCC and melanoma in that case occurred synchronously but they occurred 10 years after the AFX [14]. It was reported in literature that some cases of AFX were associated with SCC and other NMSCs but this was never reported to occur synchronously [7], [15], [16], [17], [18].

Possible causes for this multiplicity were thoroughly investigated in literature. A decrease in the cellular tolerance of U.V. radiation was demonstrated for individuals with multiple epidermal cancers. Ultraviolet light, which is the major etiology of human skin cancer, will cause mutations in the P53 gene. Such mutations occur in more than one-half of nonmelanoma squamous cell cancer and precancer. It was concluded that P53 patches, estimated to be approximately 100,000 times as common as dysplasia, have a very small or even no precancerous potential. Their common presence demonstrates that human epidermis contains a large number of P53 mutations apparently without detrimental effect. The only result of the mutation may be a ‘benign clonal keratinocytes proliferation’. So, P53 patches are frequent companions of different types of squamous cell neoplasia, but the genetic links that would support their status as early precursors of malignant neoplasia were still lacking.

Treatment modalities for primary NMSC tumours include surgical excision, radiotherapy, curettage with electrocautery, cryotherapy, laser destruction, intralesional Interferon, and some topical drug regimens. Surgical excision is the most effective treatment but renders the most tissue loss which requires functional and esthetic reconstruction in cases of head and neck cancers. Moh’s surgery may be used for large, aggressive, recurrent or location-sensitive lesions. It is the most definitive treatment but costly and time intensive. A study showed Mayo Clinic’s experience in treating AFX with Moh’s micrographic surgery (MMS) and concluded that microscopic control of the surgical margins with MMS in the treatment of AFX results in a lower recurrence rate than that with wide local excision and conserves normal tissue [19]. Radiotherapy is less effective than surgery (four-year follow-up); can cause dyspigmentation, telangiectasia and not suitable for sensitive skin as xeroderma pigmentosa patients [20], [21], [22]. Curettage with electrocautery and cryotherapy have got higher recurrence rate than surgery and radiotherapy. Also pain, leaking, and wound infection are not infrequent. Laser destruction for BCC has better cosmetic results but still with higher clinical recurrence rate than cryotherapy [20].


Conclusion

We report a case of synchronous atypical fibroxanthoma and SCC in situ (Bowen’s disease) of the head and neck in an otherwise normal old Caucasian. To the best of our knowledge this is the first case to be reported in literature with such a synchronous presentation in an otherwise normal patient. We emphasize bearing in mind the possibility of a synchronous presentation of different primary malignant lesions – especially in patients with actinic keratosis – which can help provide appropriate investigations and treatment procedures.


Notes

Competing interests

The authors declare that they have no competing interests.


References

1.
Preston DS, Stern RS. Nonmelanoma cancers of the skin. N Engl J Med. 1992 Dec 3;327(23):1649-62. DOI: 10.1056/NEJM199212033272307 Externer Link
2.
Fitzpatrick TB, Johnson RA, Wolff K, Polano MK, Suurmond D. Color atlas and synopsis of clinical dermatology: common and serious diseases. 3rd ed. New York: McGraw-Hill; 1997. p. 226:232-5.
3.
Euvrard S, Kanitakis J, Pouteil-Noble C, Dureau G, Touraine JL, Faure M, Claudy A, Thivolet J. Comparative epidemiologic study of premalignant and malignant epithelial cutaneous lesions developing after kidney and heart transplantation. J Am Acad Dermatol. 1995 Aug;33(2 Pt 1):222-9. DOI: 10.1016/0190-9622(95)90239-2 Externer Link
4.
Ko CB, Walton S, Keczkes K, Bury HP, Nicholson C. The emerging epidemic of skin cancer. Br J Dermatol. 1994 Mar;130(3):269-72. DOI: 10.1111/j.1365-2133.1994.tb02920.x Externer Link
5.
Seo SH, Shim WH, Shin DH, Kim YS, Sung HW. Pulmonary metastasis of Basal cell carcinoma. Ann Dermatol. 2011 May;23(2):213-6. DOI: 10.5021/ad.2011.23.2.213 Externer Link
6.
Helwig EB. Atypical fibroxanthoma. Proceedings of 18th Annual Tumor Seminar of San Antonio Society of Pathologists, 1961. Tex State J Med. 1961;59:664.
7.
Cooper JZ, Newman SR, Scott GA, Brown MD. Metastasizing atypical fibroxanthoma(cutaneous malignant histiocytoma): report of five cases. Dermatol Surg. 2005 Feb;31(2):221-5. DOI: 10.1111/j.1524-4725.2005.31046 Externer Link
8.
Mirza B, Weedon D. Atypical fibroxanthoma: a clinicopathological study of 89 cases. Australas J Dermatol. 2005 Nov;46(4):235-8. DOI: 10.1111/j.1440-0960.2005.00190.x Externer Link
9.
Czarnecki D, Meehan CJ, Bruce F, Culjak G. The majority of cutaneous squamous cell carcinomas arise in actinic keratoses. J Cutan Med Surg. 2002 May-Jun;6(3):207-9. DOI: 10.1007/s10227-001-0041-x Externer Link
10.
Kwon NH, Kim SY, Kim GM. A case of metastatic squamous cell carcinoma arising from actinic cheilitis. Ann Dermatol. 2011 Feb;23(1):101-3. DOI: 10.5021/ad.2011.23.1.101 Externer Link
11.
Furue M. Epithelial tumor, invasion and stroma. Ann Dermatol. 2011 May;23(2):125-31. DOI: 10.5021/ad.2011.23.2.125 Externer Link
12.
Youssef N, Vabres P, Buisson T, Brousse N, Fraitag S. Two unusual tumors in a patient with xeroderma pigmentosum: atypical fibroxanthoma and basosquamous carcinoma. J Cutan Pathol. 1999 Oct;26(9):430-5. DOI: 10.1111/j.1600-0560.1999.tb01870.x Externer Link
13.
Eckert F, Schaich B, Landthaler M. Spinozellulare Karzinome und myxoides atypisches Fibroxanthom auf aktinisch geschadigter Verbrennungsnarbe. [Spinocellular cancers and myxoid atypical fibroxanthoma of an actinically damaged burn scar]. Hautarzt. 1991 Apr;42(4):254-7.
14.
Hiscutt EL, Adams JR, Ryan JM, Langtry JA, Natarajan S. Atypical fibroxanthoma, lentigo maligna melanoma and squamous cell carcinoma arising in the site of a thermal burn treated with skin grafts. Br J Oral Maxillofac Surg. 2009 Mar;47(2):157-8. DOI: 10.1016/j.bjoms.2008.08.017 Externer Link
15.
Ferri E, Iaderosa GA, Armato E. Atypical fibroxanthoma of the external ear in a cardiac transplant recipient: case report and the causal role of the immunosuppressive therapy. Auris Nasus Larynx. 2008 Jun;35(2):260-3. DOI: 10.1016/j.anl.2007.04.013 Externer Link
16.
Nadjem MA. Case of diagnosis. Multiple atypical fibroxanthoma. Mil Med. 1986 Dec;151(12):666, 668-9.
17.
Seavolt M, McCall M. Atypical fibroxanthoma: review of the literature and summary of 13 patients treated with mohs micrographic surgery. Dermatol Surg. 2006 Mar;32(3):435-41. DOI: 10.1111/j.1524-4725.2006.32087.x Externer Link
18.
Wollina U, Schnlebe J, Koch A, Haroske G. Atypical fibroxanthoma: a series of 25 cases. J Eur Acad Dermatol Venereol. 2010 Aug;24(8):943-6. DOI: 10.1111/j.1468-3083.2010.03578.x Externer Link
19.
Davis JL, Randle HW, Zalla MJ, Roenigk RK, Brodland DG. A comparison of Mohs micrographic surgery and wide excision for the treatment of atypical fibroxanthoma. Dermatol Surg. 1997 Feb;23(2):105-10. DOI: 10.1016/S1076-0512(97)00054-X Externer Link
20.
Stulberg DL, Crandell B, Fawcett RS. Diagnosis and treatment of basal cell and squamous cell carcinomas. Am Fam Physician. 2004 Oct 15;70(8):1481-8.
21.
Rowe DE, Carroll RJ, Day CL Jr. Prognostic factors for local recurrence, metastasis, and survival rates in squamous cell carcinoma of the skin, ear, and lip. Implications for treatment modality selection. J Am Acad Dermatol. 1992 Jun;26(6):976-90. DOI: 10.1016/0190-9622(92)70144-5 Externer Link
22.
Motley R, Kersey P, Lawrence C; British Association of Dermatologists; British Association of Plastic Surgeons; Royal College of Radiologists, Faculty of Clinical Oncology. Multiprofessional guidelines for the management of the patient with primary cutaneous squamous cell carcinoma. Br J Dermatol. 2002 Jan;146(1):18-25. DOI: 10.1046/j.0007-0963.2001.04615.x Externer Link