Multiple tumour presentation of trichilemmal carcinoma 665

Multiple tumour presentation of trichilemmal carcinoma

K. O. Chan, I. J. Lim, H. G. Baladas and W. T. L. Tan

Division of Plastic Surgery, Department of Surgery, National University Hospital, Singapore

SUMMARY. Trichilemmal carcinoma is a rare skin tumour occurring in the sun-exposed areas of the elderly. Itoriginates from the external root sheath of the hair follicle and is the malignant form of the trichilemmoma.Clinically, it may be mistaken for a squamous cell carcinoma, basal cell carcinoma, nodular melanoma orkeratoacanthoma. It is distinct from the proliferating trichilemmal tumour. Trichilemmal carcinoma is usually asolitary lesion and an extensive literature search revealed no previously reported cases of multiple tumour pre-sentation. We describe a case of trichilemmal carcinoma arising from three distinct sites in the same patient anddiscuss the differential diagnoses, histological features and probable aetiology of this rare tumour. © 1999 TheBritish Association of Plastic Surgeons

Keywords: trichilemmal carcinoma, multiple tumour presentation.

Case report

A 67-year-old male Chinese shopkeeper presented with a 2-year history of a soft 1.5 cm diameter nodule in the leftsupraclavicular fossa. An excision of the lesion was per-formed based on a diagnosis of lipoma and the wound wasclosed primarily. Histology revealed the tumour to betrichilemmal carcinoma (TLC) with involvement of the mar-gins of resection. The patient, however, declined furtherresection for financial reasons and defaulted follow-up. Twoyears later, he returned with three cutaneous lesions, two ofwhich he reported had appeared within a year after excisionof the first (Fig. 1). There was a 1.5 ´ 1.0 cm plaque over theprevious excision site; one 3.5 ´ 2.5 cm polypoidal tumourover the sternal angle and another 2 ´ 1.5 cm polypoidaltumour over the right 2nd intercostal space 1.5 cm away. Thetumours were not fixed to the underlying muscle and therewas no regional lymphadenopathy. Multiple solar keratosesand Bowenoid lesions were also seen over his back, forearmsand legs. Punch biopsy of one tumour was performed whichshowed TLC. Based on this diagnosis, wide resection of allthree lesions was subsequently performed with a 1 cmmargin – the sternal and 2nd intercostal lesions beingexcised together with an intervening skin bridge. Frozensection examination of the margins confirmed adequateclearance and the resulting defects were covered with splitthickness skin grafts from the right thigh. The donor and

Figure 1—Preoperative photograph of the three cutaneous lesions.The left supraclavicular recurrence is marked with a black arrow.

recipient sites healed well, and there has been no evidence ofrecurrence to date, 18 months after the operation.

Of note in his past medical history, the patient had pul-monary tuberculosis 30 years earlier which was successfullytreated with anti-tuberculous drugs. Seven years previously,persistent haemoptysis from post-tuberculosis bronchiecta-sis necessitated a right pneumonectomy. Throughout thisperiod, he had numerous diagnostic chest X-rays (one annu-ally for follow-up for 24 years and at least 30 over the past 6years pre- and post-pneumonectomy making a total ofbetween 50 and 60 exposures) as well as a CT scan of thechest. He had otherwise remained fairly healthy, and did notreport an increased susceptibility to infections. There was nofamily history of skin malignancies, and he had not usedtraditional Chinese medications on his skin. He did notsmoke and alcohol consumption was negligible.

Histological examination of all three tumours confirmedthe diagnosis of trichilemmal carcinoma, showing charac-teristic lobules and trabeculae of atypical clear cells withvesicular nuclei and peripheral palisading extending to thedermis (Fig. 2). There was some central necrosis andsquamoid differentiation (Fig. 3). The tumour extended tothe dermis. The two chest lesions resected en bloc had a 1.5cm zone of normal skin and dermis between the tumourswith no evidence of dysplasia or carcinoma in situ.Resection margins were free of tumour.

Discussion

The term trichilemmal carcinoma was first proposedby Headington to describe ‘a histologically invasive,cytologically atypical clear cell neoplasm of adnexalkeratinocytes that is in continuity with the epidermisand/or follicular epithelium.’1 It is the malignant formof trichilemmoma and is distinct from the proliferatingtrichilemmal tumour.1 In most cases, it occurs in thesun-exposed parts of the body2 and also in patientswith xeroderma pigmentosa.3 Grossly, tumours can benodular, ulcerated or exophytic with a keratotic orcrusted appearance.3,4 The lesions have usually beenpresent for less than a year and exhibit a characteristicaccelerated growth phase, which prompts medicalconsultation.4 These tumours are locally invasive, andhave histological characteristics of high-grade mitoticpotential, however, they generally have a non-aggres-sive course and do not recur after wide excision.2–4

666 British Journal of Plastic Surgery

Figure 2—Trichilemmal tumour showing lobules of atypical cellswith peripheral palisading expanding from the surface epidermis.(Haematoxylin & eosin ´ 50.)

Figure 3—Invasive trichilemmal tumour showing nests of tumourcells with squamoid differentiation. (Haematoxylin & eosin ´ 120.)

The differential diagnoses are those of squamouscell carcinoma (SCC), pigmented basal cell carcinoma(BCC), keratoacanthoma, nodular malignant mela-noma and malignant proliferating trichilemmaltumours. The latter are nodulocystic lesions that fre-quently extend deep into the reticular dermis, areprone to local recurrence and may metastasise, unliketrichilemmal carcinoma.4

Histologically, TLC has lobular proliferation centred on a pilosebaceous unit and is composed ofcytologically malignant clear cells that are diastase-sensitive, PAS positive and exhibit trichilemmal kera-tinisation.4 The tumour may invade the dermis orintraepithelially in a Pagetoid fashion. Often, lympho-cytic and plasma cell infiltrates are seen. Some BCCmay also exhibit clear cells but there are usually areasof conventional BCC to differentiate the two. The clearcell variant of SCC does not exhibit lobular growth noradnexal extension, is PAS negative and usually has sur-rounding transitional atypia.4

The potential of ionising radiation to induce skincancer is well documented. Van Vloten et al5 found anexcess of skin cancers in patients irradiated for benigndiseases of the head and neck. There was a long latentperiod of 40 years and skin cancer prevalence wasdirectly related to radiation dose. While data on radia-tion carcinogenesis at high doses of irradiation arewell documented, there are scant data on how multiplelow doses of radiation like those incurred throughdiagnostic X-ray procedures may induce the same car-cinogenic process.5

Irradiation-induced skin cancers are mostly BCCwith a small percentage of SCC.5 Madison6 reportedtwo patients who underwent fluoroscopic-guided arti-ficial pneumothorax therapy for tuberculosis and sub-sequently developed multiple BCC on the thoracic skin26–30 years later. Myskowski et al7 described a patientwho had numerous fluoroscopic examinations fortuberculosis and about 40 years later developed multi-ple BCC over the back, as well as breast carcinoma.Solan8 similarly reported a case of multiple cancersincluding multiple BCCs, thyroid and breast carcino-mas as a result of repeated low-dose radiation expo-sure during artificial pneumothorax treatment fortuberculosis.

In our literature review, TLC has only beendescribed as a solitary lesion2–4 and has not beenreported as an irradiation-induced skin cancer. Thelocation of the tumours outside the zone of solarexposure but within the field of a standard chest X-rayfilm led us to postulate that a possible cause of thiscase of multiple tumour TLC could indeed be relatedto his prolonged period of multiple exposures to X-irradiation. This, however, does not account for thewidespread Bowenoid skin changes found on otherparts of his trunk, his arms and his legs. He also didnot appear to suffer any obvious immunocompromisesecondary to his longstanding tuberculosis.

TLC is usually a solitary indolent lesion that isclinically difficult to differentiate from other skintumours. An extensive literature search has notrevealed any previous report of a multiple tumourpresentation. It remains unclear as to whether thesetumours represent nodular recurrences or true multi-focal lesions arising from an area of unstable hair fol-licle growth. That the initial clearance was insufficientreinforces the importance of adequate margins in theresection of such lesions. Our hypothesis that thelesions may have been induced by long-term low doseirradiation remains speculative and requires a retro-spective review of similar cases for confirmation.

AcknowledgementsThe authors wish to thank Associate Professor G. C. Raju of theDepartment of Pathology, National University Hospital for hiscomments and guidance on this paper.

References

1. Headington JT. Tumors of the hair follicle: a review. Am JPathol 1976; 85: 479–514.

2. Boscaino A, Terracciano LM, Donofrio V, Ferrara G, De RosaG. Tricholemmal carcinoma: a study of seven cases. J CutanPathol 1992; 19: 94–9.

3. Reis JP, Tellechea O, Cunha MF, Baptista AP. Trichilemmalcarcinoma: a review of 8 cases. J Cutan Pathol 1993; 20:44–9.

4. Swanson PE, Marrogi AJ, Williams DJ, Cherwitz DL, WickMR. Tricholemmal carcinoma: clinicopathologic study of 10cases. J Cutan Pathol 1992; 19: 100–9.

Multiple tumour presentation of trichilemmal carcinoma 667

5. van Vloten WA, Hermans J, van Daal WAJ. Radiation-inducedskin cancer and radiodermatitis of the head and neck.Cancer 1987; 59: 411–14.

6. Madison JF. Basal cell epitheliomas after repeated fluoroscopicexaminations of the chest. Arch Dermatol 1980; 116: 323–4.

7. Myskowski PL, Gumpertz E, Safai B. Basal cell carcinoma andbreast carcinoma following repeated fluoroscopic examina-tions of the chest. Int J Dermatol 1985; 24: 122–3.

8. Solan MJ. Multiple primary carcinomas as sequelae of treat-ment of pulmonary tuberculosis with repeated inducedpneumothoraces: case report and review of the literature.Am J Clin Oncol 1991; 14: 49–51.

The AuthorsK. O. Chan MBBS, Formerly Medical Officer in Surgery, NationalUniversity Hospital, Singapore.Currently ENT Trainee, Singapore General Hospital, Singapore.

Case report

A 71-year-old obese lady presented with a large area of radi-ation dermatitis and two nodules of soft tissue recurrence ofovarian carcinoma in her lower abdominal wall. She had hadsurgical excision of an ovarian malignancy 7 years previ-ously followed by chemotherapy and radiotherapy. Fouryears later she developed an abdominal wall secondary inthe right iliac fossa, which was resected and reconstructedsuccessfully using a right tensor fascia lata flap. She had aleft knee replacement a year later. One year later she pre-sented again with two nodules in the subcutaneous planemeasuring 3 ´ 2 cm each; one of which was in the mid-lineand the other to the right of the mid-line. She also had atroublesome area of radiation dermatitis involving most ofthe left iliac fossa, which had the distorted umbilicus incor-porated in it (Fig. 1). After en bloc resection, which includedpart of the anterior rectus sheath, the lower abdominaldefect measured 25 ´ 6 cm.

An anterior thigh fasciocutaneous flap was fashioned andraised (Fig. 2). The base of the flap was 1 cm below theinguinal ligament. The superficial circumflex iliac vessel, themain vascular pedicle, was identified entering the flapmedially and this was the point of rotation.

The flap also gets additional blood supply from the supe-rior perforator of the rectus femoris and branches of the

Lower abdominal wall reconstruction using

C. E. Koshy, M. V. K. Kumar and J. Evans

Regional Plastic Surgery and Burns Unit, Derriford Hospit

SUMMARY. Full thickness defects of the lower abdominabdominal wall defects, acute trauma and following resectnot amenable to primary closure, three problems needreconstructed; (ii) stable and sensate skin coverage is needpressure; (iii) it is desirable to restore the contour of the ab

We present a case with a large area of radiation dermathe lower abdominal wall. After en bloc resection the 25 ´a sensate anterior thigh fasciocutaneous flap. The vasculareasons for using this fasciocutaneous flap in preference tAssociation of Plastic Surgeons

Keywords: abdominal wall reconstruction, thigh fasciocuta

I. J. Lim MA, MBBChir, FRCSEd, FRCS(Glasg), MMed(Surgery),Lecturer Department of Surgery, National University ofSingapore, and Registrar, Division of Plastic Surgery, Departmentof Surgery, National University Hospital, Singapore.

H. G. Baladas MBBS, FRCSEd, FRCS(Glasg), Senior RegistrarDepartment of Surgery, Alexandra Hospital, Singapore.

W. T. L. Tan MBBS, FRCSEd, FRCS(Glasg), FRACS,MMed (Surgery), FAMS (Plastic Surgery), Associate Professor andHead, Department of Surgery, National University of Singapore,and Chief, Department of Surgery, National University Hospital,Singapore.

Correspondence to Dr I. J. Lim, Division of Plastic Surgery,Department of Surgery, National University Hospital, 5 LowerKent Ridge Road, Singapore 119074.

Paper received 4 September 1998.Accepted 1 June 1999, after revision.

lateral circumflex femoral vessel, which are the two minorpedicles. The intermediate femoral cutaneous nerve withinthe flap preserved the flap sensation.

The flap was comfortably inset into the defect and thedonor site was grafted rather than closed directly although

the anterior thigh fasciocutaneous flap

al, Plymouth, UK

al wall are uncommon. They can occur in congenitalion of soft tissue tumours. In reconstruction of defects to be addressed: (i) the fascial layer needs to beed as the lower abdominal waistline area is subject todominal wall.titis and recurrence of a malignant ovarian tumour in

6 cm lower abdominal defect was reconstructed withr supply was reliable and the outcome was good. Theo the other options are discussed. © 1999 The British

neous flap.

Figure 1—Preoperative view showing the area of radiationdermatitis; there were also two soft tissue secondaries to the rightof the mid-line.