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Brown Tümör / Brown Tumor

Unusual Presentation of Brown Tumor in Lateral Malleolus And Talus, A Case Report

Lateral Malleol ve Talusta Olağandışı Yerleşim Gösteren Brown Tümörü, Vaka Sunumu

DOI: 10.4328/JCAM.2326 Received: 05.02.2014 Accepted: 15.02.2014 Printed: 01.11.2013 J Clin Anal Med 2013;4(suppl 3): 324-6Corresponding Author: H. Yener ERKEN, Anadolu Sağlık Merkezi Hastanesi Ortopedi ve Travmatoloji Kliniği, Kocaeli, Türkiye.T.: +90 2626785077 F.: +90 2626540529 E-Mail: yenererken@yahoo.com

Özet

Paratiroid bezinden parathormon’un (PTH) aşırı üretilip salgılanması primer

hiperparatirodizm’e (PHPT) yol açmaktadır. Paratiroid bezinin adenomu ya da hi-

perpazisi bu duruma yol açabilmektedir. Ortopedistler hiperparatirodizm’in (HPT),

iskelet sistem leyzonlarından, labaratuar değişkliklerinden ve tedavi seçeneklerin-

den haberdar olmadırlar. Serum kalsiyum seviyesindeki yükseklik ve HPT’nin di-

rekt grafilerdeki karakteristik özellikleri ortopedistleri bu konuda uyarmalıdır. Bu

durumlarda serumdaki intakt PTH seviyeleri ölçülmeli ve ek tanı testleri uygun

tanı için yapılmalıdır. Bu olgu sunumunda, PHPT olan 43 yaşındaki bayan hasta-

da görülen biri talus’ta diğeri lateral malleol’de 2 adet Brown tümörü sunmakta-

yız. Bu vaka, literatürde lateral malleol ve talus için bildirilmiş ilk olgu sunumu ol-

makla beraber, ek olarak ortopedistlerin bakış açısı ile HPT hakkında literatür ta-

raması yaptık.

Anahtar Kelimeler

Brown Tümör; Parathormon; Hiperparatiroidizm

Abstract

Excess production and secretion of parathormone (PTH) from parathyroid glands

causes primary hyperparathyroidism (PHPT). Orthopaedic surgeons should be

aware of skeletal manifestations, labaratory abnormalities, and the treatment

options of hyperparathyroidism (HPT). Elevated serum calcium or pathognomonic

findings of HPT on plain radiographs should alert the orthopaedic surgeon. In

these cases serum intact PTH and additional diagnostic tools should be obtained

for proper diagnosis. We report a 43 year-old patient with PHPT who developed

two Brown Tumors including one at the talus and second in the lateral malleolus.

The present case is the first report of Brown Tumor of the lateral malleolus and

talus in the literature. In additon we reviewed literature pertaining to HPT, from

orthopaedic surgeons’ aspect.

Keywords

Brown Tumor; Parathormone; Hyperparathyroidism

H. Yener Erken1, Önder Ofluoğlu2, Halil Burç3

1Anadolu Sağlık Merkezi Hastanesi Ortopedi ve Travmatoloji Kliniği, Kocaeli, 2Bahçeşehir Üniversitesi Tıp Fakültesi, İstanbul,

3Süleyman Demirel Üniversitesi Tıp Fakültesi Ortopedi ve Travmatoloji Kliniği, Isparta, Türkiye

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Introduction Excess production and secretion of the parathormone (PTH) from the parathyroid glands causes primary hyperparathyroid-ism (PHPT) which is caused by adenoma or hyperplasia of the parathyroid gland [1]. Von Recklinghausen was the first to de-scribe the characteristic bone disease of hyperparathyroidism (HPT). The Brown Tumors develop mainly in the facial bones, pelvis, ribs, and femur, and can be multifocal [2]. In this case we report a patient with PHPT who developed two Brown Tumors including one at the talus and the second in the lateral malleo-lus. To our knowledge, the present case is the first report of a Brown Tumor in the lateral malleolus and talus.

Case ReportA 43 year-old woman reported a gradual onset of pain in her left ankle lasting for 5 months. After evaluations such as plain radiographs and magnetic resonance imaging (MRI), an exci-sional biopsy was performed before.After one month, the patient described some pain and discom-fort in her ankle. Also, there was no swelling, erythema or ten-derness in the area of the previous inscision. Range of motion (ROM), stability, strength, and neurovascular examination were normal in the ankle joint. The patient was otherwise healty except for a history of pul-monary tuberculosis, which, according to the patient, had no further complications.After a physical examination, evaluations such as plain radio-graphs, and a Tc-99 bone scan were completed. Laboratory tests were normal except for calcium, phosphorous and alkaline phosphatase levels. The calcium, at 11mg/dl, was high (8.1-10.4 mg/dl), the phosphorous was slightly low at 2.3 mg/dl (2.5-5.0 mg/dl) and alkaline phosphatase was reported at 250 U/L (28-125 U/L).AP and lateral views of the cruris showed a mildly expansile lytic lesion at the distal part of the fibula close to the lateral malleo-lus (Figure 1). Although the margins of the lesion were slightly irregular, the border between the native bone and the lytic le-sion could be easily depicted. Sagittal T1 and axial T2 weighted MRI images revealed another lesion in the talus with the signal intensity identical to the fibular lesion seen on the radiography (Figure 2). Lesions had well-defined margins but were expand-ing to the peripherial soft tissues by destroying the cortex. They did not show enhancement after contrast administration, but marked perifocal enhancement was present (Figure 3). A Tc-99 bone scan demostrated increased uptake on the distal fibula, talus and 4th -7th ribs. The histologic study revealed that in the oseous sections multi-nuclated giant cells which are distributed in the stroma consist-ed of fusiform cells showing clusters and swirls are observed. Osteoid formations, some of which circumscribed with osteo-blastic rim and pigmented histiocytes were seen in the focal stromal areas (Figure 4). Because of the histologic findings, the differential diagnosis was modified to Brown Tumor of HPT (osteitis fibrosa cystica) and giant cell tumor of the bone. A serum intact PTH was ordered, and was reported as 973 pg/mL (12-72 pg/mL). After this finding, the diagnosis was confirmed as Brown Tumor of HPT. Tc 99m MIBI scans of the parathyroid glands were performed and revealed a parathyroid adenoma

Figure 1. AP and lateral views of cruris showed a mildly expansile lytic lesion at the distal part of the fibula close to the lateral malleolus.

Figure 2. (A)Sagittal and Axial MR image revealed another lesion in the talus.

Figure 4. View of the osseous specimen (Stain,hematoxylin and eosin; magnifica-tion x 10)

Figure 3. Axial MR image showed that lesions had well defined margins but were expanding to the peripherial soft tissues by destroying the cortex of the fibula(A). After contrast administration they did not show enhancement, but marked perifo-cal enhancement was present(B).

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located in the inferior right side of the thyroid gland, a multi-nodular guatr and autonomous hyperactive nodule of the thy-roid gland.

DiscussionPHPT is a relatively common disease. However, the frequency of Brown Tumor, pathognomonic skeletal form in this desease, is declining. Primary or secondary bone lesions chracterized by destructive process should be differantieted from Brown Tumor of HPT [3]. The incidence of Brown Tumor is 3% in PHPT. In secondary HPT, the incidence of Brown Tumor is 1.5% to 1.7% [3,4]. In recent years, the most commonly seen skeletal manifestation of HPT is simple diffuse osteopenia, resembling osteoporosis. The reason for the changing pattern of skeletal involvoment is unknown [5]. Although often the symptoms of osteitis fibrosa cystica are severe, the affected bone undergoes extensive rem-ineralization and healing after the removal of the parathyroid adenoma [5].In HPT, the histologic findings of the affected bone demostrates great variations. The abnormalities of the bone include osteitis fibrosa cystica (Brown Tumor) with replacement of marrow ele-ments by vascular fibrous tissue. The other abnormalities in-clude osteoporosis and osteomalacia [6].In HPT, most studies indicate cortical loss rather then trabecu-lar loss [7,8]. PTH seems to be catabolic at cortical sites and may have anabolic effects at cancellous sites of the bone. In some patients, cancellous bone density of the lumbar spine can be markedly reduced [9]. In HPT, there is a process of either bone resorption or bone formation and bone resorption is usually a dominant factor. Bone resorption is generally periarticular and is classified as subperiosteal subchondral, trabecular, endosteal, intracortical, subligamentous and subtendinous. Subperiosteal resorption is patagnomonic sign of HPT. It may be seen at any part of the body but the commonly involved parts are hands and feet [8]. The second mostly affected body part is the skull. Trabecular resorption produces a characteristic salt and paper appearance in the diploic space of the skull. Other sites of subperiosteal re-sorption include the medial aspects of the proximal tibia, femur and humerus [6,8].Bone resorption in subchondral locations are mostly in the ma-jor articulations in the axial skeleton, particularly the sacroiliac joints, sternoclavicular joints, symphisis pubis and discoverte-bral junction [10]. In several studies, differantial effects of the PTH on cancellous and cortical bone have been evaluated. It was found that the PTH affects mostly the cortical bone and the most significant postoperative improvements in bone mineral density occurs in cancellous bone [2,11]. Brown Tumors’ histopathological diagnosis include; extrava-sated blood cells areas of hemorrhage, histiocytosis including hemosiderin, trabeculation of unmineralized new bone and a mixture of osteoblasts, mononuclear cells and multiloculated giant cells [3].With current patient, plain radiographs and MRI showed an expansile cystic lesion on the lateral malleolus and talus, and an open biopsy was performed at an outside institution. One

month after this procedure, the patient visited the authors’ in-stitution. A parathyroid adenoma, multinoudular guatr and an autonomous hyperactive nodule on the thyroid gland was di-agnosed. Total excision of the parathyroid adenoma, and right total and left subtotal thyroidectomy were performed on the patient. We performed curretage and grafting for the lesions in the talus and lateral malleolus in the same session.2 years after surgery, the patient was doing well and had fully recovered. Repeat radigraphs of the ankle joint showed full healing. Labarotory values showed a normal serum calcium and intact PTH levels. The patient was able to move her ankle joint with no stiffness and had normal sensation and normal func-tion.

Competing interestsThe authors declare that they have no competing interests.

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How to cite this article:Erken HY, Ofluoğlu Ö, Burç H. Unusual Presentation of Brown Tumor in Lateral Malleolus And Talus, A Case Report. J Clin Anal Med 2013;4(suppl 3): 324-6.

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Brown Tümör / Brown Tumor