SAMT DEEL 71 16 MEI1987 665

Malignant fibrous histiocytomaof the breastA case report

C. OSTYN, I. SPECTOR, C. G. BREMNER

Summary

Primary malignant fibrous histiocytoma of the breastwas diagnosed in a 45-year-old Indian woman. Shepresented with a large ulcerated lesion and had lungmetastases. Treatment was by radiotherapy, toiletmastectomy and chemotherapy. The .patient died ofrapid extension of lung metastases 15 months afterfirst being seen. Although this tumour has previouslyb~n considered to have a relatively good prognosis,this report emphasises its fully malignant metastatic

. potential.

S Atr Med J 1987; 71: 665-666.

Malignant fibrous histiocytoma (MFH) is one of the moreunusual malignant breast tumours. It is regarded as a commonsoft-tissue sarcoma of late aduit life but few cases of primarybreast involvement have been published. Although characterisedby bizarre morphology, it is considered a potentially curablecondition if local excision is carried out early.

A case of primary breast MFH illustrating the tumour'spotential to metastasise is described.

Case report

A 45-year-old Indian woman was admined to Coronation Hospitalon 31 January 1984 with an ulcerated, bleeding tumour of the leftbreast. Eight months previously she had noticed a breast lumpwhich had grown steadily and had begun to ulcerate 4 monthsbefore. Although bleeding from the lesion had prompted her tosee.k medical anention, she also complained of dizziness, loss ofweight and a cough. She was only mildly pyrexial.

Examination revealed a hard lobular mass invading the wholeupper outer quadrant of the left breast but not fixed to thepectoralis major. The overlying skin was warm, inflamed, andulcerat~d with ~ serosanguineous discharge. There was no nippletetra.coon or discharge nor was there palpable axillary or supra­claVicular lymphadenopathy. The right breast showed noabnormalities. The patient had 5 children and was receivingcontraceptive injections. A familial history of breast disease couldnot be elicited.

H!sropathological exami?ation of a Tru-cut needle biopsyspecimen confirmed the malignant nature of the tumour. A biopsy

Department of Surgery, Coronation Hospital and Universityof the Witwatersrand, JohannesburgC. OSTYN, M.D. (LEUVEN)

C. G. BREMNER, CH.M., F.R.e.S. (ENG.), F.R.e.S. (EDIN.)

South African Institute for Medical Research and Schoolof Pathology, University of the Witwatersrand, Johannes­burg1. SPECTOR, M.B. CH.B., M.D.

specimen was also submined for oestrogen and progesteronereceptor determinations; both were negative. The haemoglobinlevel was 13,6 gldl, leucocyte count 9 300 x 10911 and alkalinephosphatase level 68 U/!. Scaphylococcus epidennidis, Escherichiacoli and Bacceroides vulgarus were cultured from the ulceratedsurface of the tumour. Liver, bone and brain scans showed noevidence of metastasis. Radiotherapy to the left breast and draininglymph node areas was gIVen (3500 rad over a 6-week period), butdespite this ~e ~ceration increased. An offensive discharge,bleedmg and dlsabhng pam led to a toilet mastectomy on 5 April1984, 1 week after completion of radiotherapy. The wound wasleft open to granulate and was successfully covered 2 weeks laterby asimple split-thickness skin graft from the thigh.

Histopathological. examination of the mastectomy specimenrevealed an MFH (Fig. 1 (a) and (b») characterised by a cartwheel(storiform) panern of variably shaped spindle cells, numerousatypical mitoses and scanered giant cells containing several hyper­chromatic irregular nuclei. Necrosis was very prominent.

Fig. 1. (a) Low- and (b) high-power views of the tumour toillusirate the storiform pattern and giant cells.

At this stage, a coin lesion (Fig. 2) became apparent in the leftlower lung lobe. Although radiologically visible at the time ofadmission, it had been largely masked by the heavy left breastshadow. Chemotherapy was started on 7 May 1984 withadriamycin, cyclophosphamide, methotrexate and vincristine. Therewas initial improvement, the mastectomy wound healed completelyand the patient gained weight. A chest radiograph on 15 July 1984showed no progression of the pulmonary metastasis, but 3 monthslater the patient complained of worsening cough and a secondlesion was found to have developed at the right lung apex. In earlyMay ~985 she was again admitted to hospital complaining of adlsablmg cough. There was no evidence of local recurrence orlymph node involvement, but she had become cachectic and asolid nodule had developed in the left lobe of the thyroid gland.Chest radiographs showed diffuse infiltration of both lung fieldsby metastatic tumour. She was discharged on symptomatic treat­ment and died at home on 16 May 1985. Permission for autopsywas refused.

Discussion

Sarcoma of the breast is uncommon (less than I%of malignantmammary lesions) and MFH exceptional. The term MFH wasfirst introduced in 1963 by Ozzello et al. I to describe amalignant soft-tissue tumour morphologically characterised bya storiform or cartwheel-like growth panern. Although the

666 SAMJ VOLUME 71 16 MAY 1987

Fig. 2. Post-mastectomy chest radiograph showing the left lungcoin lesion.

histogenesis is still the subject of some debate, there is now aclearer understanding of the behaviour of this tumour. Enzingerand Weiss2 regard it as arising from primitive mesenchymalcells showing partial histiocytic and fibroblastic differentiation.Essentially a tumour found in skeletal muscle, retroperitoneumand dermis, it has also been described in the larynx, con­junctiva, vulva, bone and breast.2 Its malignant character hasoften been underrated, although more recently several authorsH

have documented cases with distant metastases and fatal out­come. Metastases occur in almost every organ and have beendescribed in thyroid, lungs, diaphragm, heart, pericardium,spine, kidneys, adrenal glands, stomach, large and small bowel,liver, pancreas, spleen, bone, subcutaneous tissue and regionallymph nodes. 2,6 The incidence may be greater than previouslythought. In 1978, Weiss and EnzingerS emphasised that MFHwas 'the most common sarcoma of late adult life', a fullymalignant lesion with a 2-year survival rate of 60%, a recurrencerate of 44%, and a metastatic rate of 42%. Because of a poorsensitivity to radio- and chemotherapy, it has been recom­mended that this tumour be treated by 'prompt radicalsurgery'.2 Local spread beyond the gross tumour mass iscommon, and must therefore be taken into account whendetermining the necessary extent of excision. The incidence ofmetastasis to the regional lymph nodes is believed to be only12%, and routine dissection of clinically negative lymph nodesdoes not seem indicated. Tumours of the distal extremities,the most common, have a better prognosis than those of theproximal extremities or the retroperitoneum.

In a review of MFH of the breast, Langham er al. 9 noted 7previously reported cases and added 1 of their own. In 1984another 2 cases were reported by Vera-Sempere andLlombart-Bosch lO and 4 cases were reported from the MemorialSloan Kettering Cancer Center by Calleryer aLII in 1985.

The differential diagnosis of malignant histiocytic lesionsinvolving the breast includes dermatofibrosarcoma protuberans(DFP). This primary dermal lesion can invade the breasttissue and be morphologically quite similar to the storiformtype of MFH.2 But for 7 exceptional reported cases,12-15 DFPis regarded as an indolent, essentially locally malignant, non­metastasising tumour.

Radiation-induced MFH has been described after irradiationof breast carcinoma, retinoblastoma, Hodgkin's disease andmultiple myeloma. 1,10,15,16 Arising in the irradiated field severalyears after treatment, these lesions are highly malignant withrapid lethal evolution. ID

The prognosis of primary MFH of the breast is not as welldocumented. Considering it as fairly good, Langham er al. 9

discussed 4 patients whose lesions were treated by simple orradical mastectomy. There was local recurrence but no meta­stases or deaths (follow-up periods from 11 to 54 months).9The 1 patient of Vera-Sempere and Llombart-Bosch lO wasapparently free of disease 5 years after radical mastectomy. Aless favourable prognosis was found among the 5 patients withMFH diagnosed at me Sloan' Kerrering between 1949 and1982; Callery er al. 11 reported that they all presented withbreast parenchymal lesions and none had previously receivedirradiation. Two patients died of distant metasrases 13 and 22months after mastectomy (and radiotherapy after local recurr­ence); 1 patient died of an unrelated cause 23 months afterpartial mastectomy, and :? patients were apparently disease­free after 108 and 25 months respectively.

Our patient also illustrates the rapid evolution this tumourmay have. Only 24 months elapsed between the awareness of abreast lump and the death of the patient from disseminateddisease despite radiomerapy, surgery and chemotherapy. Thelocal control obtained by simple mastectomy with skin graft(after 3500 rad without clinical response) was surprisinglygood, but the distal dissemination (in lungs and thyroid)proved lethal. Chemotherapy was unable to control furtherspread. Although acceleration of the growth rate of MFH hasbeen observed during pregnancy2 suggesting hormonal depen­dency, oestrogen and progesterone receptors could not bedemonstrated in this tumour. It is therefore very unlikely thathormonal manipulation would have helped.

In summary, it would appear that primary MFH of mebreast may not be less malignant than MFH in any other site.In this patient with rapidly evolving disease, pulmonary meta­stasis was present at the time of diagnosis and, despite acombined approach of radiotherapy, surgery and chemotherapy,me patient died 15 months after first admission to hospital.

Radiotherapy was administered by the Radiotherapy Depart­ment, Hillbrow Hospital and the University of the Witwatersrand,JohannesbUJg. Chemotherapy was administered at CoronationHospital Chemotherapy Clinic by Mr R. White.

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