Turk J Med Sci2007; 37 (6): 381-385© TÜB‹TAKE-mail: medsci@tubitak.gov.tr

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CASE REPORT

Left Atrial Myxoma and Trichilemmal Cysts

Abstract: A 56-year-old man had a 6 x 5-cm mass originating from the fossa ovalis in the atrial septum foundby echocardiography and multiple enlarging scalp masses. The mass had a regular surface and was prolapsinginto the mitral valve during diastole. The tumor was successfully treated by surgical excision, which revealeda well-defined mass with a narrow-base stalk originating from the fossa ovalis in the atrial septum. Histologicalexamination confirmed the diagnosis of myxoma. The scalp masses were also removed surgically;histopathologic examination corresponded to benign trichilemmal cyst. The patient recovered withoutcomplication and was discharged 10 days after the operation.

Key Words: Myxoma, trichilemmal cyst

Sol Atriyal Miksoma ve Trikilemmal Kist

Özet: 56 yafl›nda erkek hastada kafada çok say›da kitle ve ekokardiyografide 6 x 5-cm çap›nda interatriyalseptumun fossa ovalis bölgesine tutunan kitle tespit edildi. Kalpteki kitle düzgün yüzeyli ve diyastol de solventrikül içine prolabe olmaktayd›. Kitle cerrahi olarak tamamen ç›kar›ld› ve histopatolojik de¤erlendirmemiksoma ile uyumlu olarak geldi. Kafadaki kitlelerde cerrahi olarak ç›kar›ld› ve histopatolojik de¤erlendirme detrikilmmal kist tespit edildi.Operasyon sonras› hasta sorunsuz olarak taburcu edildi.

Anahtar Sözcükler: Miksoma, trikilemmal kist

Hasan KOCATÜRK1

Mehmet Cengiz ÇOLAK2

Hikmet KOÇAK3

1 Department of Cardiology,Sifa Medical Center,Erzurum - TURKEY

2 Department of CardiovascularSurgery,Sifa Medical Center,Erzurum - TURKEY

3 Department of CardiovascularSurgery, Faculty of Medicine,Ataturk University,Erzurum - TURKEY

Received: June 12, 2007Accepted: November 15, 2007

Correspondence

Hasan KOCATÜRKDepartment of Cardiology,

Sifa Medical Center, Erzurum - TURKEY

kocaturk73@yahoo.com

Introduction

Atrial myxoma is the most common cardiac neoplasm, and up to 80% of myxomas arelocalized in the left atrium, of which 75% involve the interatrial septum. Although theyare benign mesenchymal and usually polypoid myxomatous or pedicle tumors, they maycause 3 types of clinical presentation: obstruction, embolism and constitutional (1,2).

Trichilemmal cyst (TC), a rare tumor originating from the outer root sheath of the hairfollicle, is typically seen in middle-aged or elderly patients, with a strong predilection forwomen, and is particularly localized on the scalp (3,4).

We present the case of a 56-year-old man with a left atrial myxoma and multipleenlarging scalp masses.

Case Report

A 56-year-old man presented with exercise dyspnea, and slowly growing masses onthe scalp. Dyspnea had been present for 1 year and was prominent with physical activity.The masses on the scalp had been growing slowly since he had first noticed a small lump15 years earlier. On examination, a grade 2/6 holosystolic murmur and a low mid-diastolicmurmur were audible in the apex. Pulse rate was 100 beats/min/regular and bloodpressure was 100/60 mmHg. On auscultation, the lungs were clear. Multiple masses werenoted on the scalp, ranging in size from 1-7.5 cm, although the largest was 7.5 x 6.0 cmin size and soft, immobile, fluctuating; the others were hard, non-tender, immobile andof varying diameters. The overlying skin was normal in color and texture (Figure 1). Noabnormal neurological symptoms or signs were found. Neither electrocardiogram norchest X-ray showed any abnormalities.

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Transthoracic and transesophageal echocardiographyidentified a left atrial mass, 6 cm in maximal diameter,hemodynamically similar to mitral stenosis. The tumorhad a stalk that allowed it to extend through the mitralvalve into the left ventricle during diastole (Figure 2A).The stalk of the tumor seemed to originate from theatrial septum in the fossa ovalis region (Figure 2B).Because of the localization, this mass caused mitral inflowobstruction with a maximal gradient of 14 mmHg andmean gradient of 8 mmHg (Figure 3 upper panel). A flowof mild grade mitral regurgitation along the lateral wallof the left atrium was seen by color Dopplerechocardiography. Left ventricular function anddimensions were normal.

With an assumption of tumor metastasis, contrastenhanced computed tomography (CT) of the head and

thorax was performed the same day. The CT study of thethorax showed a well-defined left atrial mass attached tointeratrial septum extending into the left ventricle (Figure4A). The CT of the head revealed multiple large,subcutaneous, hypodense solid masses with rare calcificareas.

The patient was taken to cardiac operation in order toexcise the tumor, and the gelatinous mass with a stalkattached to interatrial septum was removed with itspedicle completely (Figure 4B, Figure 5A).Microscopically, the tumor consisted of an abundantmucopolysaccharide matrix and vascular channels withvarying numbers of distinctive stellate or plump"myxoma" cells. The mucoid matrix stained positivelywith Alcian blue and mucicarmine (Figure 5B).

After the operation mitral inflow pattern was normal(Figure 3 lower panel, Figure 6)

One week after the first operation, all masses on thescalp were resected completely. The macroscopicappearance of the dominant masses appeared as firm,smooth, white-walled cysts (Figure 7A). Microscopicexamination revealed a well-circumscribed, partly cysticmass. Cysts were lined by stratified epithelium showingtrichilemmal keratinization in which the individual cellsincrease in bulk. The cells were cytologically benign. Thecystic areas were filled with trichilemmal-type keratin(Figure 7B).

The patient did well after the operation and wasdischarged on postoperative day 10. A schedule ofroutine follow-up visits was arranged to monitor therecurrence of both atrial myxoma and trichilemmal cysts.

Figure 1. Multiple scalp masses.

Figure 2. (A) Apical 2-chamber view and parasternal long-axis view showing atrial myxoma prolapsing into the left ventricle duringdiastole (LV: Left ventricle. LA: Left atrium. M: Myxoma. Ao: Aorta). (B) 2-dimensional transesophageal images clearlydemonstrating myxoma attached to interatrial septum by a distinct stalk (LA: Left atrium. M: Myxoma. Ao: Aorta. RA:Right atrium. RV: Right ventricle).

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Discussion

We herein report a case with a large left atrialmyxoma producing symptoms of mitral valve obstruction.In patients with symptoms of left heart failure, left atrialmyxoma remains an important differential diagnosis(1,2,5). As clearly seen in our case, most myxomas arisefrom the interatrial septum, with a pedicle at the borderof the fossa ovalis and prolapsing across the mitral valve

orifice. Prolapsing and polypoid myxomas have beenassociated with systemic and coronary embolism (2,5). Ahigher risk of embolization has been reported and eventsoccur in 30% to 43% of the patients (6). Our patient didnot experience any embolic event despite prolapsingmyxoma. Transthoracic and transesophagealechocardiography can generally be used to determine thelocation, size, shape, attachment and mobility (1,5,7).

Figure 3. Preoperatively continuous wave (CW)–Doppler echocardiogram revealing mitral inflowpattern similar to mitral stenosis due to flow blockage (upper panel). Normal pulsewave (PW)–Doppler of mitral inflow, postoperatively (lower panel).

Figure 4. (A) Computed tomography depicts a well-defined left atrial mass attached to interatrial septum extending into the leftventricle (LV: Left ventricle. LA: Left atrium. M: Myxoma. RA: Right atrium. RV: Right ventricle). (B) The picture obtainedduring surgery shows the myxoma (white arrow).

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Figure 5. (A) Macroscopic appearance of the resected myxoma with smooth, shiny surface (B). Microscopicappearance of cardiac myxoma shows minimal cellularity. Only scattered spindle cells with scant pinkcytoplasm are present in a loose myxoid stroma (Hematoxylin eosin stain x 100).

Figure 6. Apical 4-chamber view after the operation (LV: Leftventricle. LA: Left atrium. RA: Right atrium. RV: Rightventricle).

Figure 7. (A) Macroscopic appearance of the trichilemmal cysts. (B) Trichilemmal cyst lined by stratified squamousepithelium exhibiting trichilemmal keratinization (Hematoxylin eosin stain x 200).

Trichilemmal cyst most commonly occurs on the scalpduring the fourth to eight decade of life as a solitarylesion and is seen more frequently in women than men.They are benign lesions but rarely exhibit malignanttransformation (4,8,9). The tumor presented in this caseis worth further mention. It occurred in a man andoriginated from the scalp, the usual location for thistumor, but there were multiple masses, which has never

been reported previously. It is also surprising that carefulhistological examination did not indicate malignanttransformation despite multiple formations in size andnumber. On further examination the patient also had noevidence of metastatic disease

In this report, we describe a patient with differentbenign tumors found together incidentally.

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