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J R Soc Med. 2005 September; 98(9): 418–420.
PMCID: PMC1199640

Cold feet from adrenal leiomyosarcoma

C Wong, PhD FRCS, U O Von Oppell, PhD FCS(SA),1 and D Scott-Coombes, MD FRCS2

Among the rarer causes of cold feet is inferior vena cava thrombosis.

CASE HISTORY

A previously fit man of 57 was seen by the general physicians after acute onset of right groin pain with cold feet and altered sensation in both legs. On examination the feet were cold and cyanosed and the leg pulses were detectable only by doppler sonography. Examination was otherwise unremarkable. An ultrasound scan revealed bilateral femoral vein thrombosis and he was started on anticoagulants. A duplex scan then demonstrated thrombosis of the inferior vena cava (IVC) and both iliac veins. On abdominal CT he was found to have a massive left adrenal tumour involving the left kidney with tumour thrombus extending from the left renal vein into the IVC as far as the right atrium (Figure 1). A team including endocrine, vascular and cardiac surgeons was assembled. Additional investigations included a hormonal screen to exclude phaeochromocytoma, coronary angiography and an echocardiogram to assess the patient's fitness for open-heart surgery when a patent foramen ovale was diagnosed incidentally. He was scheduled for elective surgery but required an emergency operation after a life-threatening pulmonary embolism. The patient was placed on cardiopulmonary bypass while the endocrine surgeon excised the adrenal tumour and left kidney en-bloc. The vascular surgeon then explored the IVC; tumour thrombus was extracted and embolectomy of the venous system was undertaken. This step was followed by exploration of the right atrium, pulmonary embolectomy and closure of the foramen ovale by the cardiac surgeon. On histological examination the tumour proved to be a high-grade leiomyosarcoma of the adrenal gland.

Figure 1
Coronal reconstructions of CT scans. (a) tumour thrombus is seen extending from left renal vein to inferior vena cava (arrow); An external file that holds a picture, illustration, etc.
Object name is estar.jpg=left adrenal tumour. (b) An external file that holds a picture, illustration, etc.
Object name is estar.jpg=left adrenal tumour

After a protracted stay in the intensive care unit, with complications including septicaemia from pneumonia, arrhythmia, seizures and infection with Clostridium difficile, the patient made an excellent recovery and underwent radiotherapy. He was able to work for more than six months before the development of widespread recurrence, from which he died.

COMMENT

Leiomyosarcoma of the adrenal gland is thought to derive from the smooth muscle wall of the central adrenal vein and its branches.1 We have found nine previously reported cases, in three of which there was IVC tumour extension. The age of these patients was 30-63 years and tumour size was 11-25 cm; all were treated surgically, with extraction of tumour from the IVC.

Thrombosis of the IVC is seen in less than 2% of patients with deep vein thrombosis.2 The usual cause is propagation from iliofemoral thrombosis, consequent upon tumour extension into the IVC from renal and adrenal tumours and consequent obstruction of venous return. Thrombosis of proximal deep veins can result in an oedematous blue leg (phlegmasia caerulea dolens) and venous gangrene. About 45% of patients with IVC involvement experience obstructive symptoms.3 The tumour that most commonly invades the IVC is renal carcinoma, followed by adrenal cortical carcinoma and phaeochromocytoma.3 The incidence of IVC invasion from adrenal cortical carcinoma has been estimated at around 9%.3

The treatment of choice for mesenchymal tumours remains radical resection. For tumour thrombus that remains below the diaphragm, the IVC may be cross-clamped above the hepatic veins. For tumour thrombus that extends above the diaphragm, cardiopulmonary bypass with deep hypothermic circulatory arrest is preferred.4,5 Leiomyosarcoma is poorly responsive to adjuvant treatment. Little is known of the prognostic importance of intracaval tumour invasion from such a rare tumour: in renal cell carcinoma, provided local excision is complete, the level of caval tumour thrombus extension does not greatly influence long-term survival.6 In the present patient the major operation gave a reasonable period of survival with satisfactory quality of life.

References

1. Lujan MG, Hoang MP. Pleomorphic leiomyosarcoma of the adrenal gland. Arch Pathol Lab Med 2003;127: 32-5 [PubMed]
2. Gibbs NM. Venous thrombosis of the lower limbs with particular reference to bed-rest. Br J Surg 1957;45: 209-36 [PubMed]
3. Chesson JP, Theodorescu D. Adrenal tumor with caval extension—case report and review of the literature. Scand J Urol Nephrol 2002;36: 71-3 [PubMed]
4. Angermeier KW, Ross JH, Novick AC, Pontes JE, Cosgrove DM. Resection of nonrenal retroperitoneal tumors with large vena caval thrombi using cardiopulmonary bypass and hypothermic circulatory arrest. J Urol 1990;144: 735-9 [PubMed]
5. Marshall FF, Dietrick DD, Baumgartner WA, Reitz BA. Surgical management of renal cell carcinoma with intracaval neoplastic extension above the hepatic veins. J Urol 1988;139: 1166-72 [PubMed]
6. Moinzadeh A, Libertino JA. Prognostic significance of tumor thrombus level in patients with renal cell carcinoma and venous tumor thrombus extension. Is all T3b the same? J Urol 2004;171: 598-601 [PubMed]

Articles from Journal of the Royal Society of Medicine are provided here courtesy of Royal Society of Medicine Press