Pathologic Quiz Case: A 54-Year-Old Man With Hypertension (2003)

A 54-year-old white man underwent a routine workup for poorly controlled hypertension. The patient was otherwise asymptomatic and denied nausea, vomiting, diarrhea, flushing, abdominal pain, shortness of breath, cough, wheezing, or weight loss. A workup for a pheochromocytoma was also negative. A subsequent computed tomographic scan revealed a 7 × 6-cm, heterogeneously enhancing retroperitoneal mass beginning at the level of the renal veins and extending intrahepatically along the inferior vena cava (IVC) (Figure 1, arrowhead). The patient was referred to the biopsy service for evaluation of the abdominal mass.A fine-needle aspiration was performed under ultrasound guidance using a 22-gauge needle. The smears were air-dried and stained with Diff-Quik (Baxter, Deerfield, Ill) for on-site evaluation, as well as wet-fixed in ethanol for subsequent Papanicolaou staining. Cytopathologic examination showed numerous single cells and loosely cohesive tissue fragments. The cells were spindled, elongated, and often had long bipolar cytoplasmic processes and plump fusiform nuclei (see arrows in Figure 2, Diff-Quik, original magnification ×400). A fasciculated architecture, often with nuclear palisading, was apparent focally. A concomitantly performed core biopsy disclosed a spindle cell lesion with cigar-shaped nuclei and bright pink, eosinophilic cytoplasm. The nuclei exhibited moderate pleomorphism and hyperchromasia. Mitoses were readily identifiable (Figure 3, hematoxylin-eosin, original magnification ×400). Immunostaining for smooth muscle actin showed diffuse cytoplasmic reactivity (Figure 4, original magnification ×200).What is your diagnosis?The cytomorphologic and histopathologic appearance of the lesion in this patient is typical of leiomyosarcoma (LMS). Leiomyosarcoma shows spindled cells with plump cigar-shaped nuclei, often showing chromatic cross bars. The cytoplasm is bipolar, bright pink, and eosinophilic. The neoplastic cells are usually seen in fragments with parallel-placed nuclei, often in a fasciculated pattern. Pleomorphism, mitoses, and necrosis favor a high-grade phenotype. Immunostaining can be performed easily (using desmin and smooth muscle actin) on the cytologic material or tissue biopsy to prove myogenic origin and to differentiate these tumors from other morphologically similar entities, such as nerve sheath tumor or gastrointestinal stromal tumor. The patient underwent resection of the IVC mass and a partial hepatectomy of the caudate lobe. Grossly, the mass was 7 × 6 × 6 cm, firm, and white/yellow with a whorled cut surface showing foci of hemorrhage.Primary LMS of the great vessels is rare and most commonly arises from the IVC.12 The pathologic descriptions of IVC LMS in the literature are mostly in the form of single case reports or small series.13 To our knowledge, fine-needle aspiration findings of LMS of the IVC have not been reported previously.Most of the reported cases of IVC LMS have involved elderly women. Leiomyosarcoma of vascular origin may be extraluminal or intraluminal, or it can have both extraluminal and intraluminal components. Most cases of IVC LMS that have been described to date have been extraluminal. The tumor may arise in any part of the IVC, but most have originated from the middle third of the IVC. The site of origin of the tumor in the IVC is important because it affects when and how the patient presents, as well as the available treatment options.The clinical presentation can be highly variable, depending on the segment of the IVC involved; however, most patients present with Budd-Chiari syndrome.145 Some cases can be asymptomatic and the tumors are discovered incidentally.67 In patients with LMS originating in the upper segment of the IVC, symptoms include nausea, weight loss, and lower extremity edema. In patients with tumor involvement of the middle segment of the IVC, the symptoms may include epigastric and right upper quadrant pain, sometimes with kidney involvement and nephrotic syndrome. Arterial hypertension as a clinical presentation is also possible, as was the case in this patient.Owing to asymptomatic presentation or nonspecific symptoms, diagnosis of IVC LMS may not be made until an advanced stage. Imaging plays a pivotal role in the diagnosis of IVC LMS, and the best diagnostic modalities include computed tomographic scan and magnetic resonance imaging.36 Percutaneous needle biopsy also has been used successfully in the diagnosis of IVC LMS.5689The outcome for patients with vena caval tumors is dependent on the tumor location and the extent of metastatic spread.9 Leiomyosarcoma of the IVC does not respond well to chemotherapy or radiation. Surgical resection is the only potentially curative treatment.10 A great majority of the tumors described in the literature were resected, and prolonged survival correlated well with complete resection with negative margins. Tumor size and location are the most important prognostic indicators.1 Therefore, early diagnosis is very important and can make a difference in successful outcome. Fine-needle aspiration is a useful diagnostic modality and can be used to provide rapid diagnosis.