Intravascular leiomyosarcoma of the brachial artery: a case report

Intravascular leiomyosarcoma of the brachial artery: a case report

692 Pathology (2010), 42(7), December CORRESPONDENCE ipsilateral germ cell tumour and previous history of vasectomy. Previously hypothesised mechan...

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Pathology (2010), 42(7), December

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ipsilateral germ cell tumour and previous history of vasectomy. Previously hypothesised mechanisms for the spread of malignant germ cells to their site of arrest within vasitis nodosa have included: (1) arrest of tumour emboli within lymphatic and venous channels within vasitis nodosa following lymphovascular invasion, (2) direct pagetoid spread via the rete testis, epididymis and vas deferens, and (3) seeding of shed malignant germ cells from the testis.6 The aforementioned absence of pagetoid IGCNU involvement of the epididymis and native vas deferens epithelium in our case, together with only minimal rete testis involvement, renders the second of the above hypotheses very unlikely. This assertion is further supported by the absence to date of literature reports describing IGCNU involvement of the epididymis and native vas deferens. More recently, electron microscopy performed on IGCNU involving rete testis in a pagetoid manner has demonstrated desmosome-type cell junctions between epithelial-epithelial cells as well as epithelial-germ cells.7 The latter finding supports the theory that field effect is responsible for such pagetoid spread. Following from this, field effect seems a reasonable hypothesis for mode of spread to vasitis nodosa; however, this would need to be further investigated. In conclusion, this case serves to demonstrate an interesting and unusual manifestation of IGCNU as well as highlight the importance of thorough examination of any nodule of the vas deferens in orchidectomy specimens. Further, in combination with the phenomena of benign perineural and vascular invasion in vasitis nodosa,8–11 it raises the following questions: (1) if a high proportion of cases of IGCNU within the testes progress to invasive germ cell tumours,12 could the same apply for IGCNU involving vasitis nodosa; and (2) if so, in men with malignant germ cell tumours as well as vasitis nodosa, could the latter potentially be an additional site from which the germ cell tumour may spread? T. T. Khamu* G. M. Strutton* A. Kiosoglous{ Departments of *Anatomical Pathology, Pathology Queensland, and {Urology, Princess Alexandra Hospital, Brisbane, Queensland, Australia Contact Dr T. T. Khamu. E-mail: [email protected]

1. Olson AL. Vasitis nodosa. Am J Clin Pathol 1971; 55: 364–8. 2. Gru¨ner OP. Regenerative growth of the vas deferens resembling a lowgrade carcinoma. Report of a case. Scand J Urol Nephrol 1970; 4: 83–5. 3. Civantos F, Lubin J, Rywlin AM. Vasitis nodosa. Arch Pathol 1972; 94: 355–61. 4. Lee AH, Theaker JM. Pagetoid spread of intratubular germ cell neoplasia into the rete testis. Histopathology 1994; 24: 385–9. 5. Perry A, Wiley EL, Albores-Saavedra G. Pagetoid spread of intratubular germ cell neoplasia into rete testis: a morphologic and histochemical study of 100 orchidectomy specimens with invasive germ cell tumors. Hum Pathol 1994; 25: 235–9. 6. Heaton JM, Maclennan KA. Vasitis nodosa – a site of arrest of malignant germ cells. Histopathology 1986; 10: 981–9. 7. Mai KT, Yazdi HM, Rippstein P. Light and electron microscopy of the pagetoid spread of germ cell carcinoma in the rete testis: morphologic evidence suggestive of field effect as a mechanism of tumor spread. Appl Immunohistochem Mol Morphol 2001; 9: 335–9.

8. Balogh K, Travis WD. Benign vascular invasion in vasitis nodosa. Am J Clin Pathol 1985; 83: 426–30. 9. Balogh K, Travis WD. The frequency of perineurial ductules in vasitis nodosa. Am J Clin Pathol 1984; 82: 710–3. 10. Zimmerman KG, Johnson PC, Paplanus SH. Nerve invasion by benign proliferating ductules in vasitis nodosa. Cancer 1983; 51: 2066–9. 11. Goldman RL, Azzopardi JG. Benign neural invasion in vasitis nodosa. Histopathology 1982; 6: 309–15. 12. Woodward PJ, Mostofi FK, Talerman A, et al. Germ cell tumours. In: Eble JN, Sauter G, Epstein JI, et al. World Health Organisation Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs, Lyon: IARC Press, 2004; 228–30.

DOI: 10.3109/00313025.2010.523688

Intravascular leiomyosarcoma of the brachial artery: a case report Sir, While leiomyosarcoma is a relatively common soft tissue malignancy, primary vascular leiomyosarcoma is rare. So far less than 300 cases have been reported.1 These cases were five times more commonly of venous origin than of arterial origin,2 with the majority arising from the inferior vena cava or from larger veins of the lower limbs. Of the tumours arising within arteries, over 50% originated in the pulmonary artery. Other less commonly reported sites include the aorta, renal, popliteal, splenic, subclavian, carotid, iliac, common femoral and superficial femoral arteries,2 with the majority of these arising within the popliteal artery.3 To the best of our knowledge, no cases of vascular leiomyosarcoma arising from the upper limb arteries have thus far been reported. Here we present a case of primary leiomyosarcoma arising from a branch of the brachial artery. A 67-year-old female presented to her local doctor complaining of a lump within her mid-upper left arm. Clinical examination at the time revealed a semi-firm 30 mm mass abutting the right median nerve which was not fixed by flexion and was suspected to be either a lipoma or neurofibroma. Surgical exploration at a provincial hospital revealed a 30 6 25 mm saccular mass arising from a muscular artery immediately distal to its origin from the right brachial artery. The mass was 10 cm above the left elbow within the intramuscular compartment, adjacent to the efferent vein and closely abutting (but was not tethered to) the left median nerve. Macroscopic examination revealed a 30 6 22 6 20 mm cream, bosselated and partially encapsulated nodule with a 5 mm length of attached vessel at one margin. The cut surface appeared white-cream and whirled with occasional haemorrhagic foci and a 5 mm softer, clear, gelatinous region towards the centre. Histologically, this malignant tumour was composed of fascicles of neoplastic spindle cells with eosinophilic cytoplasm and elongated blunt-ended nuclei (Fig. 1A) which showed significant nuclear pleomorphism, frequent often bizarre mitoses (23–20 per high power field) and multinucleation (Fig. 1B). Focal areas of hyalinisation and myxoid change with occasional areas of necrosis were also present. The tumour was present within a medium sized blood vessel (Fig. 2A). Histochemical studies using orcein

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giemsa (Fig. 2B) revealed the tumour to be arising from smooth muscle cell within the wall. At the outer margin the tumour was partially encapsulated by a thin layer of dense collagen.

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Immunohistochemical stains performed found the neoplastic cells to be diffusely positive for smooth muscle actin (SMA) (Fig. 3A), with patchy positivity for desmin (Fig. 3B). The cells were negative for S-100, CK 5/6,

Fig. 1 Histological examination of the tumour. (A) Fascicles of neoplastic spindle cells with eosinophilic cytoplasm and elongated blunt-ended nuclei. (B) Significant nuclear pleomorphism with frequent bizarre mitoses and multinucleation (H&E).

Fig. 2 (A) Histological examination revealed the tumour was arising from smooth muscle cells of the wall of this medium sized artery (H&E). (B) Elastic stain confirms presence of the artery (Orcein Giemsa).

Fig. 3

Immunohistochemical studies of the tumour. (A) SMA; (B) desmin; (C) Ki-67 study for proliferation index.

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pan-cytokeratin, HMB45, EMA, CD34 and CD117. The proliferation index (Ki-67 positive cells) was 70% (Fig. 3C). These morphological features and this immunoprofile were consistent with the diagnosis of an intermediate grade leiomyosarcoma arising from the wall of a medium sized vessel and these findings correlated with the surgical findings of the lesion arising from a proximal branch of the left brachial artery. The patient subsequently underwent wider local re-excision which revealed a small area of residual leiomyosarcoma but clear surgical margins. The patient underwent adjuvant radiotherapy and at 15 month follow up was disease free. Soft tissue tumours make up 0.7% of all malignancies, of which leiomyosarcomas (non-gynaecological) account for 5–7%.4 Leiomyosarcomas can be subdivided into four main groups: intra-abdominal (40–45%); subcutaneous/ deep soft tissue (30–35%); cutaneous (15–20%); intravascular (5%),5 of which only one-sixth are arterial in origin, occurring most commonly in adults, with no gender predilection.2 Tumours of arterial origin have a tendency to be more aggressive than those of venous derivation,2 and these tumours can be further divided prognostically by whether they arise from the intima, which tends to exhibit poorer differentiation and higher potential for direct spread, or the media, which tends to be better differentiated with less tendency to seed directly.6 As in our case, the tumours typically present as white to tan fleshy masses with a whorled pattern on the cut surface, often with areas of haemorrhage, necrosis or cystic change.7 Microscopically, they typically exhibit intersecting bundles of spindle cells with blunt-ended ‘cigar-shaped’ nuclei, which may show invagination or multilobation, are usually hyperchromatic and pleomorphic (even if only mildly) and contain frequent, often atypical, mitosis.7 The cytoplasm ranges from pale to eosinophilic and may show vacuolation or fibrillary change, and there may be associated focal fibrosis, myxoid change, hyalinisation, microcytic change or coagulative necrosis.7 The immunoprofile of these tumours shows uniform strong positivity for SMA and/or desmin (70–80%) and caldesmon (60–65%),5 with negative staining for S-100, HMB-45, EMA, pancytokeratin, CD34, CD56 and CD117. If there is any doubt as to the diagnosis, electron microscopy may be used to show the classical nuclear morphology of smooth muscle cells, with cytoplasmic filaments with densities, cell junctions, pinocytotic vesicles and basement membrane.7 Vascular leiomyosarcomas can be graded using the French Federation of Cancer Centres System (FFCCS) grading scheme for adult sarcomas, which uses tumour differentiation, mitotic rate and tumour cell necrosis, and staged using the American Joint Committee on Cancer (AJCC) staging system for soft tissue sarcoma, which incorporates local anatomical site and extent, metastatic status and histological grade.5 Prognosis of these tumours depends on stage at presentation and surgical resectability, with metastasis occurring in 60% of patients, primarily to lungs, liver and lymph nodes.2 Advanced age (i.e., older than 60 years), vascular invasion and DNA aneuploidy have also been identified as factors conferring a worse prognosis in these tumours.8 Treatment of choice is

surgical resection where possible, and Abed et al. found that good local control could often be achieved with en bloc surgical resection of vessels in combination with radiotherapy.9 However, even with this, the 5 year survival of these patients is 36%, with an average survival rate of 16 months.4 In summary, we present the case of rare vascular tumour arising in a unique location. Vascular leiomyosarcomas are defined as leiomyosarcomas that arise from a vessel and this diagnosis is based on the combination of the clinical picture, radiographic images and pathological findings.9 Our case demonstrates just such an exceptional case, with a histologically confirmed leiomyosarcoma identified surgically to arise from a previously unreported site, a major branch of the right brachial artery. Vanathi Sivasubramaniam Min Ru Qiu Department of Anatomical Pathology, Sydpath, St Vincent’s Hospital, Sydney, NSW, Australia Contact Dr Vanathi Sivasubramaniam. E-mail: [email protected] Acknowledgements: The authors would like to thank Associate Professor Phil Allen, SouthPath, Flinders Medical Centre, Adelaide, South Australia.

1. Hong SA, Hong ME, Kwon GY, Lee TJ, Park ES, Yoo JH. Intravascular leiomyosarcoma of the femoral vein – a case report. Korean J Pathol 2008; 42: 232–5. 2. Sakpal SV, Mehta R, Babel N, Chamberlain RS. Peripheral artery leiomyosarcoma. J Vasc Surg 2009; 49: 217–21. 3. Tilkorn D-J, Lehnhardt M, Hauser J, et al. Intravascular leiomyosarcoma of the brachiocephalic region – report of an unusual tumour localisation: case report and review of the literature world. J Surg Oncol 2008; 6: 113. 4. Cardarelli GF, Barellini L, Faviana P, Guerra M. Leiomyosarcoma of the popliteal artery: case report and review of the literature. J Vasc Surg 2003; 37: 206–9. 5. Fletcher CDM. Diagnostic Histopathology of Tumors. 3rd ed., New York: Churchill Livingstone, 2007. 6. Szekely E, Kulka J, Miklos I, Kaliszky P. Leiomyosarcoma of great vessels. Pathol Oncol Res 2000; 6: 233–6. 7. Fletcher CDM, Unni KK, Mertens F. World Health Organization Classification of Tumours. Pathology and Genetics of Soft Tissue and Bone. Lyon: IARC Press, 2003; 131–4. 8. Giangola G, Migaly J, Crawford B, Moskowitz P, Sebenick M. Leiomyosarcoma of the subclavian artery. J Vasc Surg 1995; 22: 496– 500. 9. Abed R, Abudu A, Grimer RJ, Tillman M, Carter SR, Jeys L. Leiomyosarcoma of vascular origin in the extremity. Sarcoma 2009; 385164: (Epub Jun 29).

DOI: 10.3109/00313025.2010.523688 523691

Myelinoid bodies in a patient with membranoproliferative glomerulonephritis Sir, We present a case of myelinoid bodies seen on electron microscopy in renal disease following a previous