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the fifth and sixth decades. Most times the pulmonary In our patient, we postulate that a fistula was created
AVF is single, abuts the pleura, and is found in the lower between the right interlobar pulmonary artery and an
lungs. Classified as either simple or complex, up to 90% adjacent branch of the inferior pulmonary vein by the
are the former, with all feeding arteries arising from a automated stapling device used during wedge resection
single subsegmental artery. Causes include hepatopul- of the coccidioma. This complication gave rise to exer-
monary syndrome, metastatic thyroid carcinoma, schis- tional dyspnea, hypoxemia, and paradoxical embolism,
tosomiasis, actinomycosis, necrotizing pneumonias, mi- and the fistula was identified on cardiac magnetic reso-
tral stenosis, systemic amyloidosis, penetrating chest nance imaging and computed tomographic angiography,
trauma, and surgery for lung resection or congenital and as it was too large to treat safely with embolization,
cardiac conditions [5]. so it was surgically resected. There are no other reports of
Pulmonary AVFs do not usually affect cardiac hemo- an AVF after wedge resection of a lung lesion with a
dynamics. The degree of right-to-left shunt and the stapling device.
potential for paradoxical embolism dictate the clinical
manifestations [5]. Patients may present with an asymp-
tomatic lung nodule on a chest roentgenogram or with References
one or more of a broad spectrum of symptoms, including 1. Kim EA, Lee KS, Shim YM, et al. Radiographic and CT
epistaxis, dyspnea, and hemoptysis. Physical examina- findings in complications following pulmonary resection.
tion may reveal cyanosis, digital clubbing, or pulmo- Radiographics 2002;22:67– 86.
nary vascular bruits. Complications are often neuro- 2. McHugh K, Hedge V, Brock P, Elliott MJ. Bronchial artery to
bronchial vein fistula secondary to lung nodule resection.
logic, secondary to paradoxical emboli. These include Arch Dis Child 2005;90:384.
transient ischemic attacks, strokes, brain abscesses, 3. Riehl G, Chaffanjon P, Frey G, Sessa C, Brichon PY. Postop-
migraine headaches, and seizures. Other complica- erative systemic artery to pulmonary vessel fistula: analysis of
tions include hemothorax resulting from transpleural three cases. Ann Thorac Surg 2003;76:1873–7.
rupture, hemoptysis, polycythemia, anemia, and pul- 4. Stedman’s Medical Dictionary, 27th edit. Baltimore, MD:
Lippincott, Williams & Wilkins, 2000:679.
monary hypertension. 5. Gossage JR, Kanj G. Pulmonary arteriovenous malformations:
The diagnostic evaluation is centered on identifying its A state of the art review. Am J Respir Crit Care Med
presence as well as determining treatment. Oxygen sat- 1998;158:643– 61.
uration studies nearly always demonstrate hypoxemia 6. Khurshid I, Downie GH. Pulmonary arteriovenous malforma-
and an elevated shunt fraction (⬎5%) [5]. The chest tion. Postgrad Med J 2002;78:191–7.
FEATURE ARTICLES
roentgenogram is usually abnormal in symptomatic pa-
tients, and it may reveal single or multiple round or oval
masses of uniform density, occasional lobulation, sharply PEComa (Clear Cell “Sugar” Tumor)
defined borders, and tubular opacities representing feed- of the Lung: A Benign Tumor That
ing vessels [5]. Contrast-enhanced computed tomogra-
phy is useful for diagnosis, and pulmonary angiography
Presented With Trombocytosis
provides further anatomical detail for consideration of Serdar Sen, MD, Ekrem Senturk, MD,
embolization. Contrast echocardiography, radionuclide Nilgun Kanlıoglu Kuman, MD, Engin Pabuscu, MD,
perfusion lung scanning, and magnetic resonance imag- and Firuzan Kacar, MD
ing can also be helpful. Contrast echocardiography can Departments of Thoracic Surgery and Pathology, Adnan
detect a right-to-left shunt, but it does not provide Menderes University, Faculty of Medicine, Aydin, Turkey
anatomical detail. Perfusion lung scanning may reveal
decreased pulmonary arterial perfusion of the involved
Perivascular epithelioid cell tumors of the lung are rare, be-
lobe and unexpected radionuclide activity in the brain
nign neoplasms, usually presenting as a solitary pulmonary
and kidneys. The pulmonary AVF can be seen on a
nodule on chest roentgenograms. Most lesions are solitary
magnetic resonance image, but computed tomographic
and asymptomatic, and are located within the peripheral
angiography is preferred, due to its higher spatial
lung. This is a case report of a 44-year-old woman who
resolution.
presented with thrombocytosis and solitary pulmonary
Treatment of the pulmonary AVF encompasses either
surgery or embolotherapy. An untreated pulmonary AVF nodule of the lung, which was removed by a thoracotomy.
may lead to considerable morbidity and mortality. Treat- (Ann Thorac Surg 2009;88:2013–5)
ment is indicated when it is symptomatic, greater than 2 © 2009 by The Society of Thoracic Surgeons
cm in diameter, or has feeding arteries at least 3 mm in
diameter [5]. Surgical resection was the mainstay of
treatment until the late 1970s. Percutaneous embolization P erivascular epithelioid cell tumor (PEComa) (Clear
cell “sugar” tumor) of the lungs is a rare, pulmonary
lesion that was originally described by Liebow and
using coils or balloons (when feasible) is now the treat-
ment of choice, because it preserves lung parenchyma, is
less invasive, and can be repeated if necessary [6]. A large Accepted for publication May 13, 2009.
or central AVF may not be suitable for embolization due Address correspondence to Dr Senturk, Adnan Menderes University
to the high risk of distal embolization to the left-sided Medical Faculty, Department of Thoracic Surgeon, Aydin, 09100, Turkey;
circulation. e-mail: ekremsenturk@hotmail.com.
its intense post-contrast enhancement on computed tomo- the patient underwent the operation. The elastic tumor was
graphic scans, this tumor may simulate a malignant neo- reached through a pneumotomy and was enucleated from
plasm, such as primary or metastatic lung cancer. Histo- the lung parenchyma. The tumor weighed 16 g and mea-
logic and immunohistochemical findings differentiate this sured 4 ⫻ 3 cm in diameter; the tumor was white in color
tumor from the metastatic renal cell carcinoma [6]. and had bleeding areas shown in the macroscopic exami-
Transthoracic fine-needle biopsy is a valuable diagnos- nation (Fig 2A). A rich blood supply was evident in many
tic method of diagnosis, especially in a suspected malig- parts of the tumor.
In the microscopic examination, eosinophilic cyto- uL before the operation, and has been dramatically
plasm and diffuse growth pattern were determinate in all decreased to level of 758,000 uL after the resection on
sections. Tumor cells staining with periodic acid-Schiff/ postoperative day ten. Until now, we have not described
distaste periodic acid-Schiff positive, with musine nega- any PEComa case that had been presented with throm-
tive. Immunohistochemically, these were stained with bocytosis in the literature. We believed that the cause of
s100 diffuse and with HMB45 focally (Figs 2B–D). There is thrombocytosis that had been considered as essential by
no staining with cytoceratin and CD68. The vascular hematologists was PEComa. We believed that the renal
structures are determinate by vimentin and CD34. The cell tumor could be presented with thrombocytosis, but
histopathologic diagnosis is reported as the clear cell there was no pathologic finding from the abdominal and
(sugar) tumor of lung (ie, PEComa). pelvic ultrasonography. There was an increase found in
Thrombocyte counts were 853,000 UL, 795,000 mL, and thrombocytosis among individuals with ovarian malig-
768,000 UL on postoperative days 2, 4, and 10, respec- nancy, with as great as 33% of those with ovarian germ cell
tively. No complication or recurrence occurred in the tumors demonstrating preoperative thrombocytosis [10].
postoperative follow-up period. Definitive diagnosis of PEComa can be obtained by
transthoracic tissue biopsy or open biopsy through a
Comment thoracotomy. Others have stated that the resection of
tumor was enough for the treatment [2, 3, 11]. In our
PEComa (clear cell “sugar” tumor) is an unusual, benign, case, the radiologic diagnoses were sclerosing heman-
pulmonary tumor that was first described by Liebow and gioma and malignancy. The transthoracic biopsy was
Castleman [1] in 1971, and resembles the renal cell not advised because of the suspicion of hemangioma.
carcinoma both theoretically and practically. Liebow and The diagnostic and therapeutic thoracotomy and enu-
Castleman [1], reported a study that included 12 cases. cleation were performed after the frozen section was
There were no significant findings from the physical investigated.
examinations of their patients, and none of the tumors Perivascular epithelioid cell tumor of the lung is a
could be visualized by bronchoscopy. Radiographic visu- benign tumor that is rarely seen, and it would be pre-
alization revealed that the tumors were rounded or sented with symptoms such as headaches, weakness, and
ovoid, smoothly countered, peripheral parenchymal essential thrombocytosis as with our patient.
masses. The microscopic appearance through stained
FEATURE ARTICLES
sections (by hematoxylin and eosin) was distinctive and
rather uniform [1]. References
The reported cases each presented as a solitary, pul- 1. Liebow AA, Castleman B. Benign clear cell (“sugar”) tumors
monary nodule, and all of them were asymptomatic, of the lung. Yale J Biol Med 1971;43:213–22.
except only one case that was reported as a mass [4, 8]. 2. Shimosato Y, Miller RR. Biopsy interpretation of the lung.
The tumor was usually detected accidentally on roent- New York: Raven Press, 1995.
genogram as a circumscribed peripheral mass [7]. Den- 3. Folpe AL. Neoplasms with perivascular epithelioid cell dif-
ferentiation (PEComas). In: Fletcher CD, Unni KK, Mertens
sitometry measurements should be performed on medi- F, ed. World health organization classification of tumors.
astinal sections to minimize partial-volume effects. These Pathology and genetics of tumors of soft tissue and bone.
should also be obtained by placing regions of interest to Lyon: IARC Press, 2002:221–2.
occupy approximately 70% of the short-axis and long- 4. Policarpio-Nicolas ML, Covell J, Bregman J, Atkins K. Fine
axis dimension of the lung nodule on computed tomo- needle aspiration cytology of clear cell “sugar” tumor (PE-
Coma) of the lung: report of a case. Diagn Cytopathol
graphic image [7]. Swensen and colleagues [8] demon- 2005;36:91–3.
strated 98% sensitivity and 58% specificity for benign 5. Nguyen GK. Aspiration biopsy cytology of benign clear cell
results using less than 15 Hounsfield Units (HU) as the (“sugar”) tumor of the lung. Acta Cytol 1989;33:511–5.
maximal amount of enhancement from pre-contrast im- 6. Santana ANC, Nunes FS, Ho N, Takagaki TY. A rare cause of
ages. In the study by Swensen and colleagues [9], nodule hemoptisis: benign sugar (clear) cell tumor of the lung. Eur
J Cardiothorac Surg 2004;25:652– 4.
dimensions ranged between 5 and 40 mm; however, most 7. Gora-Gebka A, Liberek W, Bako M, Szumera M, Korzon K,
of the mean diameters of the nodules were greater than Jaskiewicz K. The “sugar” clear cell tumor of the lung—
10 mm (mean, 14 mm and 17 mm for benign and clinical presentation and diagnostic difficulties of an unusual
malignant nodules, respectively). lung tumor in youth. J Pediatr Surg 2006;41:27–9.
Our patient complained of headaches and weakness, 8. Swensen SJ, Morin RL, Schueler BA, et al. Solitary pulmonary
nodule: CT evaluation of enhancement with iodinated contrast
and the essential thrombocytosis was defined with the material—a preliminary report. Radiology 1992;182:343–7.
hematologic examination. No pathologic finding was 9. Kula O, Bekci TT, Senol T, Pazarli P. Akcigerin iyi Huylu
found to explain the cause of thrombocytosis, which can Clear Cell Tumoru. Akciger Arsivi 2002;2:67–9.
have prognostic significance in lung cancer and is asso- 10. Ferrigno D, Buccheri G. Hematologic counts and clinical
ciated with poor outcome in patients with adenocarci- correlates in 1201 newly diagnosed lung cancer patients.
Monaldi Arch Chest Dis 2003;59:193– 8.
noma and epidermoid cancers [9]. In our case, the throm- 11. Haddad LB, Laufer MR. Thrombocytosis associated with
bocyte count was decreasing regularly after the tumor malignant ovarian lesions within a pediatric/adolescent
resection. The level of thrombocyte count was 1,075,000 population. J Pediatr Adolesc Gynecol 2008;21:243– 6.