- Email: [email protected]
Spontaneous rupture of adrenocortical carcinoma
The American Journal of Surgery 194 (2007) 77–78
Clinical image
Spontaneous rupture of adrenocortical carcinoma Koichi Suyama, M.D.a, Toru Beppu, M.D., Ph.D.a, Takatoshi Isiko, M.D., Ph.D.a, Shin-ichi Sugiyama, M.D.a, Katsutaka Matsumoto, M.D.a, Koichi Doi, M.D., Ph.D.a, Toshiro Masuda, M.D.a, Chitoshi Ohara, M.D., Ph.D.b, Hiroshi Takamori, M.D., Ph.D.a, Kei-ichiro Kanemitsu, M.D., Ph.D.a, Masahiko Hirota, M.D., Ph.D.a, Hideo Baba, M.D., Ph.D.a,* a
Department of Gastroenterological Surgery, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-8556, Japan b Department of Surgery, National Hospital Organization Kumamoto Saisyunso Hospital, Kumamoto, Japan Manuscript received May 12, 2006; revised manuscript October 5, 2006
Abstract Massive hemorrhage from an adrenocortical carcinoma seldom occurs in the retroperitoneal or abdominal cavity. We report a case of spontaneous rupture of primary adrenocortical carcinoma occurring in an adolescent. A right adrenalectomy with complete removal of the tumor was performed successfully. © 2007 Excerpta Medica Inc. All rights reserved. Keywords: Adrenocortical carcinoma; Acute abdomen; Spontaneous rupture
A 17-year-old female with a 1-year history of right upper abdominal pain was shown to have a right adrenal tumor measuring 13 ⫻ 9 cm by magnetic resonance imaging but she refused surgery. One year later, she experienced the sudden onset of severe upper-abdominal pain. Computed tomography showed an increase in tumor size to 15 ⫻ 12 cm with intratumoral and peritumoral blood (Fig. 1). Emergency surgery was performed. The tumor compressed the liver, kidney, duodenum, and inferior vena cava, but did not invade these organs (Fig. 2). The tumor was removed completely. The final histologic diagnosis was adrenocortical carcinoma. There were no lymph node metastases. The postoperative course was uneventful and the patient was discharged 11 days after the surgery. No adjuvant chemotherapy was administered. The patient is alive and diseasefree 48 months after tumor resection. About 60% of adrenocortical carcinomas are functional and produce endocrine symptoms [1,2]. Modern imaging techniques have made it possible to detect many asymptomatic adrenal tumors. Most nonfunctional tumors are large when they are detected, as in this instance. Adrenocortical carcinoma develops in patients of all ages but is most frequent in the very young (⬍5 y) and in the fourth to fifth
* Corresponding author. Tel.: ⫹81-96-373-5213; fax: ⫹81-96-3714378. E-mail address: [email protected]
Fig. 1. Enhanced abdominal computed tomography showing a huge mass with an enhanced capsule. (A) plain phase; (B) arterial phase; (C) delayed phase. The diameter was increased to 15 ⫻ 12 cm, compared with 9 ⫻ 7 cm 14 months previously. IVC, inferior vena cava; Ao, aorta.
decade of life [2]. Bleeding into the tumor and rupture of the tumor are rare events. Typical radiographic features of a malignant adrenal tumor are large size (⬎5 cm), vague border, irregular shape, and heterogenous texture [1]. Complete resection of the tumor and adjacent lymph nodes is required for cure. The development of intratumoral necrosis is an important prognostic factor of adrenocortical carcinoma. It is difficult to distinguish adenoma from carcinoma on histologic criteria. The diagnosis of carcinoma is supported by the presence of intratumoral necrosis or bleeding, large tumor size, evidence of local invasion, high mitotic count, and/or distant metastasis [3,4]. The overall survival rate in patients with adrenocortical carcinoma is calculated based on 3 of those risk factors [4]: high mitotic count, larger
0002-9610/07/$ – see front matter © 2007 Excerpta Medica Inc. All rights reserved. doi:10.1016/j.amjsurg.2006.10.028
78
K. Suyama et al. / The American Journal of Surgery 194 (2007) 77–78
with all 3 risk factors [4]. Our patient had 2 risk factors, but she remains alive without recurrence 48 months after the surgery. To our knowledge, this is the ninth case of a spontaneous rupture of an adrenocortical carcinoma described in the literature.
Fig. 2. Intraoperative findings. (A) A huge tumor compressing the liver and the right kidney was observed in a retroperitoneal cavity. (B) A tumor that had a strong capsule and fibrous adhesion to the duodenum and the inferior vena cava was recognized.
size, and intratumoral hemorrhage. Patients with no risk factors have an 83% 5-year survival rate, decreasing to 42% with 1 risk factor, 33% with 2 risk factors, and 0%
References [1] Joao R, Raul CR, Barry DF. Imaging findings in pediatric adrenocortical carcinoma. Pediatr Radiol 2000;30:45–51. [2] Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol 2003;169:5–11. [3] Geoffrey A. Adrenocortical neoplasms in children: radiologic-pathologic correlation. Radiographics 1999;19:989 –1008. [4] Harrison E, Paul B, Murray F. Pathologic features of prognostic significance for adrenocortical carcinoma after curative resection. Arch Surg 1999;34:181–5.
Clinical image
Spontaneous rupture of adrenocortical carcinoma Koichi Suyama, M.D.a, Toru Beppu, M.D., Ph.D.a, Takatoshi Isiko, M.D., Ph.D.a, Shin-ichi Sugiyama, M.D.a, Katsutaka Matsumoto, M.D.a, Koichi Doi, M.D., Ph.D.a, Toshiro Masuda, M.D.a, Chitoshi Ohara, M.D., Ph.D.b, Hiroshi Takamori, M.D., Ph.D.a, Kei-ichiro Kanemitsu, M.D., Ph.D.a, Masahiko Hirota, M.D., Ph.D.a, Hideo Baba, M.D., Ph.D.a,* a
Department of Gastroenterological Surgery, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-8556, Japan b Department of Surgery, National Hospital Organization Kumamoto Saisyunso Hospital, Kumamoto, Japan Manuscript received May 12, 2006; revised manuscript October 5, 2006
Abstract Massive hemorrhage from an adrenocortical carcinoma seldom occurs in the retroperitoneal or abdominal cavity. We report a case of spontaneous rupture of primary adrenocortical carcinoma occurring in an adolescent. A right adrenalectomy with complete removal of the tumor was performed successfully. © 2007 Excerpta Medica Inc. All rights reserved. Keywords: Adrenocortical carcinoma; Acute abdomen; Spontaneous rupture
A 17-year-old female with a 1-year history of right upper abdominal pain was shown to have a right adrenal tumor measuring 13 ⫻ 9 cm by magnetic resonance imaging but she refused surgery. One year later, she experienced the sudden onset of severe upper-abdominal pain. Computed tomography showed an increase in tumor size to 15 ⫻ 12 cm with intratumoral and peritumoral blood (Fig. 1). Emergency surgery was performed. The tumor compressed the liver, kidney, duodenum, and inferior vena cava, but did not invade these organs (Fig. 2). The tumor was removed completely. The final histologic diagnosis was adrenocortical carcinoma. There were no lymph node metastases. The postoperative course was uneventful and the patient was discharged 11 days after the surgery. No adjuvant chemotherapy was administered. The patient is alive and diseasefree 48 months after tumor resection. About 60% of adrenocortical carcinomas are functional and produce endocrine symptoms [1,2]. Modern imaging techniques have made it possible to detect many asymptomatic adrenal tumors. Most nonfunctional tumors are large when they are detected, as in this instance. Adrenocortical carcinoma develops in patients of all ages but is most frequent in the very young (⬍5 y) and in the fourth to fifth
* Corresponding author. Tel.: ⫹81-96-373-5213; fax: ⫹81-96-3714378. E-mail address: [email protected]
Fig. 1. Enhanced abdominal computed tomography showing a huge mass with an enhanced capsule. (A) plain phase; (B) arterial phase; (C) delayed phase. The diameter was increased to 15 ⫻ 12 cm, compared with 9 ⫻ 7 cm 14 months previously. IVC, inferior vena cava; Ao, aorta.
decade of life [2]. Bleeding into the tumor and rupture of the tumor are rare events. Typical radiographic features of a malignant adrenal tumor are large size (⬎5 cm), vague border, irregular shape, and heterogenous texture [1]. Complete resection of the tumor and adjacent lymph nodes is required for cure. The development of intratumoral necrosis is an important prognostic factor of adrenocortical carcinoma. It is difficult to distinguish adenoma from carcinoma on histologic criteria. The diagnosis of carcinoma is supported by the presence of intratumoral necrosis or bleeding, large tumor size, evidence of local invasion, high mitotic count, and/or distant metastasis [3,4]. The overall survival rate in patients with adrenocortical carcinoma is calculated based on 3 of those risk factors [4]: high mitotic count, larger
0002-9610/07/$ – see front matter © 2007 Excerpta Medica Inc. All rights reserved. doi:10.1016/j.amjsurg.2006.10.028
78
K. Suyama et al. / The American Journal of Surgery 194 (2007) 77–78
with all 3 risk factors [4]. Our patient had 2 risk factors, but she remains alive without recurrence 48 months after the surgery. To our knowledge, this is the ninth case of a spontaneous rupture of an adrenocortical carcinoma described in the literature.
Fig. 2. Intraoperative findings. (A) A huge tumor compressing the liver and the right kidney was observed in a retroperitoneal cavity. (B) A tumor that had a strong capsule and fibrous adhesion to the duodenum and the inferior vena cava was recognized.
size, and intratumoral hemorrhage. Patients with no risk factors have an 83% 5-year survival rate, decreasing to 42% with 1 risk factor, 33% with 2 risk factors, and 0%
References [1] Joao R, Raul CR, Barry DF. Imaging findings in pediatric adrenocortical carcinoma. Pediatr Radiol 2000;30:45–51. [2] Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol 2003;169:5–11. [3] Geoffrey A. Adrenocortical neoplasms in children: radiologic-pathologic correlation. Radiographics 1999;19:989 –1008. [4] Harrison E, Paul B, Murray F. Pathologic features of prognostic significance for adrenocortical carcinoma after curative resection. Arch Surg 1999;34:181–5.