Malignant Nodular Hidradenoma: a Case Report1

J Korean Radiol Soc 2007;56:579-583

Malignant Nodular Hidradenoma: A Case Report1

Jin Hwan Kwon, M.D., Jin Do Huh, M.D., Kyung Soon Jeong, M.D., Mi Hee Jung, M.D., Ji Ho Ko, M.D., Jae Do Kim, M.D.2, Bong Kwon Chun, M.D.3, Seon-Joo Lee, M.D.4

Malignant nodular hidradenoma is a rare skin appendageal tumor, and its imaging findings have not been previously described. We experienced the case of a large malignant nodular hidradenoma of the left upper arm in a 71-year-old woman. MRI revealed a large, lobular, poorly circumscribed, soft tissue mass at the left upper arm, and the mass showed homogeneous enhancement. 18F-FDG PET/CT showed hyper- metabolic activity in the left upper arm mass with a maximal standard uptake value of 19.

Index words : Skin, neoplasms Skin, MR Skin, radionuclide studies

Skin appendageal tumors are generally diagnosed ac- cording to their histology, and imaging examinations are Case Report not routinely performed. However, when a large soft tissue mass exists and malignancy is suspected, then imag- A 71-year-old woman presented with a recurrent tu- ing may be helpful to scrutinize the nature of the tumor. mor of the left upper arm. The lesion was first noted 50 Malignant nodular hidradenoma is a rare aggressive ma- years earlier as a small, bean-sized, cutaneous nodule, lignant tumor of the eccrine sweat glands. and it had been stable for 46 years. About 4 years before Approximately 50 cases had been documented up to the year 2004 in the English literature, and most cases have been reported in the pathology literature with only lim- ited clinical and radiological information (1). We present here the radiological findings of a malignant nodular hidradenoma of the upper arm in a 71-year-old woman.

1Department of Diagnostic Radiology, Gospel Hospital, College of Medicine, Kosin University 2Department of Orthopedic Surgery, Gospel Hospital, College of Medicine, Kosin University 3Department of Pathology, Gospel Hospital, College of Medicine, Kosin University 4Department of Diagnostic Radiology, Busan Paik Hospital, College of Medicine, Inje University Received March 9, 2007 ; Accepted April 18, 2007 Address reprint requests to : Jin Do Huh, M.D., Department of Fig. 1. A 71-year-old woman with malignant nodular hidrade- Diagnostic Radiology, Gospel Hospital, College of Medicine, Kosin noma. A photograph of the malignant nodular hidradenoma University, 34 Amnam-dong, Seo-Gu, Busan 602-702, Korea Tel. 82-51-990-6341 Fax. 82-51-255-2764 showed a 12×10×4 cm sized ulcerated fungating mass that E-mail: [email protected] involve the left upper arm. ─ 579 ─ Jin Hwan Kwon, et al : Malignant Nodular Hidradenoma

admission, the nodule began to slowly grow and the patient was treated at a private clinic. An incisional biopsy was then performed and the diagnosis of benign sweat gland tumor was made. Two years later, the nodule began to rapidly enlarge with ulceration and hemorrhagic discoloration. At the time of admission, a superficial 12 ×10×4 cm mass lesion was palpated on the postero-lateral aspect of the left upper arm (Fig. 1). The tumor was elastic and firm and it was not associated with any mo- tor or sensory disturbance. Plain radiographs demonstrated a lobular soft tissue

Fig. 2. Conventional radiography showed a multilobular soft Fig. 4. The fused PET-CT image showed an area of hyperme- tissue mass at the distal part of the upper arm. No bony abnor- tabolism (arrow, Standard Uptake Value=19) corresponding mality in the adjacent humerus was found. to the soft tissue mass in the left upper arm.

A B C Fig. 3. The axial T1-weighted (A) and fat-suppressed T2-weighted (B) images showed an ill defined mass (arrow) in the subcutaneous tissue of the upper arm. The mass was hypointense to muscle on the T1-weighted image and hyperintense to muscle on the fat-suppressed T2-weighted image. The axial fat-suppressed T1-weighted image (C) after intravenous administration of gadolinium demonstrated homogeneous enhancement (arrowhead). The underlying adjacent musculature was involved.

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A B Fig. 5. A. Microscopic examination of the tumor revealed proliferation of two types of cells, basoloid or squamoid cells and clear cells, and they formed a lobular pattern and also solid sheets (hematoxylin and eosin stain, magnification ×200). B. A hidroadenoma with areas of malignant transformation was observed. Note the dispersed growth pattern with local invasion (hematoxylin and eosin stain, magnification ×200).

mass with no internal calcification. No definite change such as erosion, periosteal reaction or invasion was not- Discussion ed in the adjacent bone (Fig. 2). The MR images showed a soft tissue mass in the subcutaneous layer of the lateral Malignant nodular hidradenoma is a rare aggressive epitrochlear region, and the mass extended to the adja- malignant tumor of the eccrine sweat glands. Several cent muscular layer. The tumor’s margin was poorly de- synonyms for malignant nodular hidradenoma have ap- fined and the lesion was nearly homogenous in appear- peared in the literature: clear-cell hidradenocarcinoma, ance. The mass showed an intermediate to low signal in- malignant clear-cell hidradenoma, solid-cystic adenocar- tensity on the T1-weighted images, and near uniform in- cinoma, malignant acrospiroma, malignant clear-cell termediate to high signal intensity on the fat-suppressed myoepithelioma and clear-cell eccrine carcinoma (1). T2-weighted images. On the fat-suppressed T1-weighted This tumor’s incidence has been reported to be 6% of all MR images with contrast enhancement, the mass the eccrine gland carcinomas (2). The tumor usually pre- demonstrated diffuse enhancement without any areas sents as a solitary painless papule or nodule, with a slow of necrosis (Fig. 3). The fused PET-CT showed hyperme- growing course, on the head, trunk or distal extremities, tabolic activity in the mass with a maximal standard up- and sometimes there are multiple tumors (3). The usual take value of 19. This finding was suggestive of a malig- age of occurrence is older than 50 years with an equal nant lesion. No other sites of abnormal focal FDG up- sex distribution. The prognosis for the 5-year survival af- take were apparent on the whole-body PET-CT scan ter excision is less than 30% (1, 3). Malignancy can oc- (Fig. 4). cur de novo in normal skin or it can develop within a The patient underwent an excisional biopsy and split- pre-existing benign eccrine tumor (4-6). Considering thickness skin grafting. The histopathologic study the very long history of our patient, we believe the tu- showed an ill-defined, epithelial neoformation that was mor started as a benign tumor and it then turned into a formed by lobules of clear polygonal cells at both the malignant one. deep dermis and the subcutaneous tissue. There was a Histopathogically, the tumor cells usually have char- second group of smaller cells that had a basaloid aspect, acteristic vacuolated cytoplasm (clear-cell change) ow- and a few of them showed slight atypia (Fig. 5). The fi- ing primarily to glycogen accumulation; however, an- nal pathologic analysis confirmed the diagnosis of malig- other possibility is that such cells are expressing my- nant nodular hidradenoma. No evidence of lymph node oephithelial differentiation, based on the focal coexpres- metastases or distant metastases was found. sion of keratin and actin in some tumor cells (1, 3). Another characteristic feature of malignant nodular ─ 581 ─ Jin Hwan Kwon, et al : Malignant Nodular Hidradenoma hidradenoma is the presence of ductal lumina lined by tive in controlling recurrence or metastatic tumors. epithelial cells or eosiophilic cuticles, and these struc- Chemotherapy has been used sporadically for residual tures are histolgically analogous to the eccrine sweat metastatic disease, although its value has not been con- duct (1). firmed (1, 3, 6). Any reported MRI findings of benign hidradenoma In summary, malignant nodular hidradenoma typical- are extremely rare, and only a few cases have been re- ly affects adult patients as a painless mass of the extrem- ported on. Reier et al. (7) reported on a plantar eccrine ities at the time of presentation. Malignant nodular acrospiroma that was 1 cm in diameter, and this re- hidradenoma may manifest on MR images as a multi- vealed a solid enhancing nodule. Maldjian et al. (8) re- lobular and poorly circumscribed mass with homoge- ported that clear cell hidradenoma showed a complex nous enhancement that involves the adjacent muscle. cystic appearance with fluid levels that suggested hem- Despite the relative rarity of this tumor, it should be in- orrhage and there were enhancing mural soft tissue nod- cluded in the differential diagnosis when finding subcu- ules. Tsurumaru et al. (9) reported that clear cell taneous masses in the extremities of adult patients. hidradenoma showed a solid and cystic appearance with fluid levels and an enhancing solid component. To References the best of our knowledge, the MRI appearance of malignant nodular hidradenoma has not been previously 1. Ohta M, Hiramoto M, Fujii M. Nodular hidradenocarcinoma on the scalp of a young woman: case report and review of literature. described. The tumor in our case was revealed to be a Dermatol Surg 2004;30:1265-1268 large sized, multilobular and poorly circumscribed mass 2. Wu H, Elenitsas R. Malignant nodular hidradenoma in a patient with homogeneous enhancement. The cut surface of the with neurofibromatosis type 1: a case report and review of the lit- resected specimens showed solid nests of neoplastic erature. Cutis 2001;68:273-278 3. Waxtein L, Vega E, Cortes R, Hojyo T, Dominguez-Soto L. cells that featured basoloid and clear cells along with Malignant nodular hidradenoma. Int J Dermatol 1998;37:225-228 with higher cellularity, and this corresponded to the ho- 4. Galadari E, Mehragam AH, Lee KC. Malignant transformation of mogenous enhancement on the contrast-enhanced MR eccrine tumours. J Cutan Pathol 1987;14:15-22 images. 5. Lim SC, Lee MJ, Lee MS, Kee KH, Suh CH. Giant hidradenocarcinoma: a report of malignant transformation from nodular hidrade- The value of the MR in this case was therefore similar noma. Pathol Int 1998;48:818-823 to that in most soft tissue tumors, that is, to help define 6. Khalil HMB, Yusuf H, Kaddour HS. Malignant eccrine hidradeno- the extent of the tumor and to aid in the planning of ma of neck causing acute heart failure. Auris Nasus Larynx surgery rather than providing a specific preoperative di- 2003;30:307-310 7. Reier AR, Farooki S, Ashman CJ, Miles L. MR imaging appearance agnosis of the tumor. In our case, PET-CT has been of plantar eccrine acrospiroma (sweat gland tumor). AJR Am J shown to be useful in differentiating malignant from be- Roentgenol 2002;179:1079-1080 nign lesions and for excluding distant metastasis. 8. Maldjian C, Adam R, Bonakdarpour A, Robinson TM, Shienbaum AJ. MRI appearance of clear cell hidradenoma. Skeletal Radiol The best treatment to achieve cure is wide and deep 1999;28:104-106 excision. Recurrence is common after incomplete exci- 9. Tsurumaru D, Torii Y, Kajiwara T, Shimoda Y, Yoshikane K, Irie sion. Metastases typically appear first in the regional K, et al. MRI of clear cell hidradenoma. Radiat Med 2004;22:426- lymph nodes, lung and bone. Radiotherapy is not effec- 428

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대한영상의학회지 2007;56:579-583

악성 결절성 한선종: 증례 보고1

1고신대학교 복음병원 영상의학과, 2정형외과, 3해부병리과 4인제대학교 의과대학 부산백병원 영상의학과

권진환·허진도·정경순·정미희·고지호·김재도 2·천봉권 3·이선주 4

악성 결절성 한선종은 매우 드문 피부 부속기 종양으로 알려졌다. 저자들은 71세 여자환자의 좌측 상완에 발생 한 악성 결절성 한선종 증례를 경험하였기에 영상학적 소견을 보고한다. 병변은 자기공명영상에서는 경계가 잘 그 려지지 않고 균등한 조영증강을 보이는 연부조직종양으로 관찰되었다. 18F-FDG PET/CT에서는 18F-FDG의 높 은 섭취증가를 보이며 SUV는 19로 측정되었다.

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