Journal of the Royal Society of Medicine Volume 85 April 1992

carcinoma occurring in rhinophymatous skin and that ofthe population at large. Dysplastic areas were present at the lateral excision margins in our patient suggesting an unstable, premalignant phase. Brubaker reported a case where clear histological progression from hyperplasia to carcinoma was seen5. However, the case for recognizing rhinophyma as a premalignant condition remains a poor one and is generally unaccepted. Most skin carcinomata arise as a result of actinic damage which is usually limited to the most superficial layers. The hidden nature of the tumour seen in our case suggests that invasion occurred into the deeper, subcutaneous layers at a relatively early stage which may explain the extensive occult infiltration at the time of presentation. In a recent case report, a sebaceous carcinoma was discovered quite unexpectedly on routine histological analysis following paring of a chronic rhinophyma which had been noted to undergo a rapid increase in size; further emphasizing the case for care when assessing these supposedly innocuous lesions6. Our case, along with those

A case of malignant epithelioid schwannoma arising on the face

D A Ross BSc FRCS K Schafler FRCPath Regional Plastic Surgery Unit, Queen Mary's University Hospital, Roehampton, London SW15 Keywords: malignant schwannoma; malignant epithelioid schwannoma

Malignant schwannoma is a rare but well recognized tumour of peripheral nerve and neurofibromas accounting for approximately 10% of soft tissue sarcomas. Diagnostic difficulty may occur when the tumour does not appear to arise from a nerve or is poorly differentiated. In this situation the tumour shares certain histological features with malignant melanoma. One may also have to consider a number of even rarer variants such as malignant epithelioid schwannoma. Case report A 74-year-old Caucasian woman presented with a 3 year history of a nodular, non-pigmented lesion at the base ofthe right alar. The appearance suggested a basal cell carcinoma.

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discussed above, would suggest long standing rhinophyma may be susceptible to malignant change and that close attention is warranted to any associated lesions. A small lesion may be the 'tip of the iceberg', References 1 Wolfe MM. Rhinophyma; with new aetiological and therapeutic considerations. Laryngoscope 1943;53:172-80 2 Freeman BS. Reconstructive rhinoplasty for rhinophyma. Plast Reconstr Surg 1970;46:265-70 3 Acker DW, Helwig EB. Rhinop with carcinoma. Arch Dermat 1967;95:250-4 4 Broadbent NRG, Cort DF. Squamous cell carcinoma in longstanding rhinophyma. Br J Plastic Surg 1977;31:308-9 5 Brubaker DB, Hellstom RH. Carcinoma arising in rhinophyma. Arch Dermatol 1977;113:847-8 6 Motley RJ, Douglas-Jones AF. Sebaceous carcinoma: an unusual cause of a rapidly enlarging rhinophyma. Br J Dermatol 1991;124:283-4 (Accepted 18 June 1991)

Histology revealed a tumour composed of pallisades of spindle cells but no evidence of junctional melanocyte activity. The tumour did not arise from a preexisting peripheral nerve lesion nor were there any stigmata of neurofibromatosis. Thirty-two months later she represented with an area of ulceration on the rim of the right alar. Biopsy confirmed recurrence and reexcision necessitated the removal of both soft tissue and the anterior wall of the right maxilla. Histology showed tumour to have invaded the right infraorbital nerve (Figure 1), which was resected at the floor of the orbit. A final procedure, through the floor ofthe orbit, succeeded in removing the residual tumour and she remains well with no signs of recurrence at 6 months.

Case presented to Section of Plastic Surgery, 8 January 1991

Discussion Malignant schwannoma is a rare tumour but well recognized in association with peripheral nerve tumours, especially von Recklinghausen's disease'. In the apparent absence of a preceding nerve lesion, diagnosis of these tumours may be exceedingly difficult and require ultrastructural and immunohistochemical studies. Differential diagnoses comprise malignant melanoma, poorly differentiated carcinoma, lymphoma and rare variants. These include the malignant 'Triton' tumour2, glandular malignant schwannoma3, and malignant epithelioid schwannoma. Malignant epithelioid schwannoma has been chronicled in several series in association with peripheral nerves and arising below the clavicles4, but descriptions of lesions developing on the head and neck are considerably more rare and confined to individual case reports. The mean age ofpresentation in these reports was 71 years. Three ofthe four reported cases, including our patient, were female. As in two other reports our patient presented with a nodular, unpigmented lesion on the face5'6. In the third case the tumour arose in the mandible of a 27-year-old

Ethiopian woman7.

Figure 1. Low power view ofthe tumour (H&E, x25) Note the spindle celled configuration on the left and a more solid epithelioid tumour island on the right

The histology of the initial specimen from our patient revealed several classical features of this tumour. In certain areas epithelioid cells are apparent, some multinucleate, and these were more prevalent in the recurrent tumour, a feature noted before'. Histology of the infraorbital nerve in the recurrent specimen clearly showed the tumour to be invading it as opposed to arising from it (Figure 2), and consequently it falls into the rare category of malignant epithelioid schwannoma developing in soft tissue'. In certain areas the tumour cells merged with the perineurium of small nerves

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Journal of the Royal Society of Medicine Volume 85 April 1992

Figure 2. This section shows tumour invading the infraorbital nerve (H&E, x250)

and with the desmoplastic stroma at the tumours edge. This feature can lead to serious difficulty for the pathologist, especially if the surgeon is relying on the results of frozen section to assess tumour clearance. Major differential diagnoses are distinguished by stains, immunohistochemistry and electron microscopy. Immunohistochemical use of keratin markers and leucocyte common antigen will detect the carcinomas and lymphomas. However, S100 positivity which is found mainly in neural crest derived tumours will therefore not distinguish between malignant schwannoma and malignant melanoma. The similarity between these two tumours was initially reported by Reed and Leonard8, who described a variant of desmoplastic melanoma which shared certain histological features with malignant epithelioid schwannoma. Their case, however, involved a pigmented lesion arising in an area of lentigo. Enzinger and Weiss further clarified the picture when, in 1983 they described a 'malignant epithelioid schwannoma of soft tissue origin'. They too recognized the similarity between this tumour and malignant melanoma but defined it as a schwannoma on the basis of two key features; firstly the absence of melanocyte activity within the epidermo-dermal junction and secondly the absence of melanin pigment'. Electron microscopy has revealed other features to aid distinction including a distinct basal lamina. The most important however, is the absence of melanosomes or pre-melanosomes characteristic of melanoma9. The natural history of those tumours arising below the clavicles suggests a virulent tumour. In a study of 14 patients nine died, six from metastatic disease within 2 years

CMV colitis in an immunocompetent adult

S D Blair MS FRCS A Forbes MD MRCP R A Parlins MD FRCP Gastrointestinal Unit, Charing Cross Hospital, London W6 8RF Keywords: cytomegalovirus; colitis; immunocompetent; foscarnet

Mild cytomegalovirus (CMV) infection is common, but very rarely causes colitis in immunocompetent adults. We report the first UK case of CMV colitis in the immunocompetent. The patient, the fourth case in the world literature to survive, is the first immunocompetent individual in whom the antiviral agent foscarnet has been used therapeutically.

of diagnosis. Spread occurred via both haematogenous and lymphatic routes with the low incidence of local recurrence being attributed to radical excision of the primary lesion4. As indicated reports of tumours arising on the head and neck are few and one has to be guarded in characterizing its behaviour. Of three reported cases, two died of metastatic disease 3 and 6 years after presentation57. Significantly both patients had prolonged problems with locally recurrent disease necessitating repeated attempts at excision prior to rapid spread of the tumour and death. In the case reported by Bleach5, radical surgery was not carried out initially due to the patient's wishes, however repeated excision resulted in considerable morbidity. Radiotherapy did not provide any additional benefit. Chemotherapy was given in Chu's report7, but only at an advanced stage (the author does not give any details of the regimen used). The third case was well at 3 years follow-up with no evidence of spread6. However, in the light of experience ofboth our case and those above, one may debate as to whether the patient was diseasefree. Furthermore, prolonged follow-up would be essential. Experience from those cases discussed suggests that radical excision is indicated to achieve local control. Follow up will then be required over a 5 year period or longer. References 1 Enzinger FM, Weiss SW. Soft tissue tumours. St Louis: CV Mosby, 1983:625-56 2 Daimaru Y, Hashimoto H, Enjoji M. Malignant 'Triton' tumours: a clinico-pathologic and immunohistochemical study of nine cases. Hum Pathol 1984;15:768-78 3 Woodruff JM, Chernick NL, Smith MC, Millett WB, Foote FW. Peripheral nerve tumours with rhabdomyosarcomatous differentiation. Cancer 1973;32:426-39 4 Lodding P, Kindblom LG, Angervall L. Epithelioid malignant schwannoma, a study of 14 cases. Virchows Arch 1986;409-.433-51 5 Bleach NR, Keen CE, Dixon JA. Superficial malignant schwannoma on the face: a case for early radical surgery. J Laryngol Otol 1989;103:316-18 6 Morgan K, Gray C. Malignant epithelioid schwannoma of soft tissue? A case report with immunohistology and electron microscopy. Histopathology 1985;9:765-75 7 Chu T, Shmookler BM. Malignant epithelioid schwannoma: a light microscopic and immunohistochemical study. JSurg Oncol 1988;39:68-72 8 Reed RJ, Leonard DD. Neurotropic melanoma. A variant of desmoplastic melanoma. Am J Surg Pathol 1979;3:301-11 9 Taxy JB, Battifora H, Trujillo Y, Dorfman HD. Electron microscopy in the diagnosis of malignant epithelioid schwannoma. Cancer 1981;48:1381-91

(Accepted 1 October 1991)

Case report A 35-year-old previously fit man presented with a 9 day history of increasing diarrhoea, malaise and nightsweats. Loose stools of small volume, with mucus but no blood were passed up to 12 times a day. On examination, he was unwell but not dehydrated, with a pyrexia of 39.5°C. Abdominal examination including sigmoidosoopy was normal. An initial diagnosis of infective diarrhoea was made, and he was started on codeine, ampicillin and metronidazole. Stool cultures remained negative and colonoscopy was performed. This showed five large almost circumferential areas of superficial ulceration between the sigmoid and hepatic flexure. There was minimal reaction at the edges and no nodularity; the rest of the mucosa was endoscopically normal. Histology of the ulcers showed granulation tissue including scattered large cells with intranuclear and cytoplasmic inclusions which stained for cytomegalovirus antigen by the immunoperoxidase technique. There was otherwise no focal active inflammation

Case presented to Clinical Section, 10 May 1991

0141-0768/92/ 040238-02/S02.00/0 © 1992 The Royal Society of Medicine

A case of malignant epithelioid schwannoma arising on the face.

Journal of the Royal Society of Medicine Volume 85 April 1992 carcinoma occurring in rhinophymatous skin and that ofthe population at large. Dysplast...
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