Poland′s syndrome is a rare congenital anomaly characterized by chest wall and upper limb abnormalities. Some lymphreticular and solid malignancies have been reported with this syndrome. Renal cell carcinoma associated with Poland′s syndrome has not been described previously. We herein describe the first case of a Poland′s syndrome associated with renal cell carcinoma diagnosed incidentally after a road accident. All the cases with Poland′s syndrome should be aware of onchologic attention by the radiologists and clinicians.

Introduction

Poland′s syndrome is a rare congenital anomaly consists of absence or hipoplasia of the pectoralis major muscles, malformation of the upper limbs, breasts and nipples, ribs and costal cartiages, chest wall subcutenous tissues. This syndrome may be associated with hypoplasia of the subclavian artery or its branches which may lead to different developmental changes. [1] Cases of Poland′s syndrome associated with lymphoreticular malignencies, [2] and solid tumors as leiomyosarcoma, [3] neuroblastoma, [4] Wilm′s tumor, [5] breast cancer, [6] lung cancer, [7] and gastric cancer, [8] were reported. These may confirm the relationship between developmental defects and tumors and require oncologic attention.

To our knowledge, we herein describe the first case of a Poland′s syndrome associated with renal cell carcinoma diagnosed incidentally after a road accident. The aim of this article is to evaluate the multidetector computed tomography (MDCT) findings of Poland′s syndrome and draw the attention of the radiologists and clinicians in these cases may have increased risk of malignancies.

Case Report

A 51-year-old unconscious patient after a road accident came to our department for the evaluation of the thorax with chest radiography and MDCT. On supine anteroposterior chest radiography left hemithorax was slightly hiperlusent. The MDCT examinations were performed on a eight channel MDCT system (GE Medical Systems, Milwaukee, WI, USA). MDCT revealed pleural effusion, compression atelectasis, minimally deplaced and fragmented fractures of the ribs II to V on the right hemithorax. On the left hemithorax, the pectoralis major and minor, subscapularis, supraspinatus, infraspinatus, serratus anterior, teres major and minor muscles were absent. Erector spina and trapezius muscles were hypoplastic [Figure 1]. These findings confirmed the presence of Poland′s syndrome. In the abdominal sections, a lobulated and heterogenously enhancing mass with cystic areas at the right kidney was observed [Figure 2]. The mass was extended to the perirenal fat planes and renal sinus. The imaging appearance of the renal mass suggested renal cell carcinoma. The patient underwent radical nephrectomy at another institution. Pathological examination revealed renal cell carcinoma.{Figure 1}{Figure 2}

Discussion

Poland′s syndrome was first described by Alfred Poland, in 1871. The incidence of the syndrome is between 1:7000 and 1:100,000. It is a nongenetic, congenital disorder. The features of the syndrome show differences. It is mostly unilateral. The major components of the syndrome are the absence of sternocostal head of pectoralis major and complete absence of pectoralis minor muscles. Varying degrees of congenital malformations including, the other chest wall and upper limb muscles, breast, nipple and areola, rib cage, hand, and phalanges. Also skeletal, genitourinary, cardiac, and endocrine abnormalities have been associated. In the pathogenesis of the Poland′s syndrome, the defective development of the blood supply of the subclavian, internal thoracic, and brachial arteries are accused. Poland′s syndrome may be associated with Möbius and Klippel-Feil syndomes. These two syndromes are due to abnormalities of the primitive trigeminal arteries and the vertebral arteries, respectively. [1]

This is the first case of Poland′s syndrome associated with renal cell carcinoma. There is an association between Poland′s syndrome and malignancy. Lymphoreticular malignancies, [2] and solid tumors as leiomyosarcoma, [3] neuroblastoma, [4] Wilm′s tumor, [5] breast cancer, [6] lung cancer, [7] and gastric cancer [8] in the cases with Poland′s syndrome were reported. We, as the other authors assume that Poland′s syndrome is a congenital disorder associated with increased risk of lymphreticular and solid malignancies. The association between some congenital syndromes and malignancies is known. Down′s syndrome and Bloom′s syndrome have increased risks of leukemia. Also the associated syndromes with Poland′s syndrome, as mentioned above, also have increased risks for the development of some tumors. Klippel-Feil syndrome has a risk for the development of the posterior fossa dermoid cysts [9] and Möbius syndrome has a risk for the development of the Wilms′ tumor. [10]

MDCT is an important examination method for the trauma patients in emergency departments as our patient. The unconscious trauma patients underwent MDCT, so that multiple systems are evaluated. However, MDCT is commonly using in radiology practice for the detection of the chest. When the clinicians are suspected about a lung disease, for example unilateral hiperlucent lung at radiography, as our patient, the second imaging method is MDCT. Thus, the radiologists are commonly encountering with developmental abnormalities of the chest. Especially for radiologist, it is important to know the association between this syndrome and various tumors.

Therefore, all the examinations of the cases with Poland′s syndrome should be carefully detected for a possible malignancy by the radiologist, and the incidental cases with Poland′s syndrome should be aware of onchologic attention by the radiologists and clinicians.