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Adrenal myelolipoma. A case report
Ann Urol 2002 ; 36 : 95-8 2002 Éditions scientifiques et médicales Elsevier SAS. Tous droits réservés S0003-4401(01)00080-8/SCO
Surrénales
Adrenal myelolipoma. A case report A. Benchekroun ∗ , H. Jira, M. Ghadouane, E.H. Kasmaoui, M. Zannoud, M. Faik Department of urology A, Ibn Sina hospital Rabat, Morocco
ABSTRACT We report a new case in a 56 years old patient who manifested right side’s pains. The abdominal CT scan showed a tumor of the right adrenal gland with fat density (size: 8 × 6 × 6 cm). This tumor was removed surgically (weight: 300 g). The histological finding revealed a proliferation of adipocyts and myeloid tissue which contains megakaryocytes, erythroid cells and lymphocyts. The outcome is good after three years of surgery. The surgical treatment becomes necessary when the tumor’s size increases or it becomes symptomatic. 2002 Éditions scientifiques et médicales Elsevier SAS adrenal gland / myelolipoma / surgery
RÉSUMÉ Myélolipome de la surrénale. À propos d’un cas. Le myélolipome de la surrénale est une tumeur rare et bénigne. Nous rapportons une nouvelle observation chez un patient âgé de 56 ans qui présentait des douleurs du flanc droit. La tomodensitographie (TDM) abdominale avait montré une tumeur surrénalienne droite de densité graisseuse mesurant 8 × 6 × 6 cm. Une tumeur de 300 g a été enlevée chirurgicalement. L’histologie a montré une prolifération d’adipocytes et un tissu myéloïde contenant des mégacaryocytes, des érythrocytes et des lymphocytes. L’évolution a été bonne avec trois ans de recul. Le traitement chirurgical du myélolipome de la surrénale s’avère nécessaire lorsque celui-ci devient symptomatique ou augmente de taille. 2002 Éditions scientifiques et médicales Elsevier SAS chirurgie / myélolipome / surrénale
(Received 9 April 2001; accepted 4 May 2001)
∗ Correspondence and reprints.
1. INTRODUCTION The myelolipoma (ML) of adrenal gland is a benign and non functionning tumor that is composed of mature adipose tissue and hematopoietic elements similar to those seen in the bone marrows [1, 6, 9]. It’s a rare affection since until 1999, only 300 cases have been reported in world literature [2, 9]. We report another case and call back the characteristics of this pathologic entity.
2. CASE REPORT Mr. B.H., 56 years old, without particular pathology’s history, was examinated in our institution for right side’s pains in 1996. The clinical exam in first was normal. Then the abdominal CT scan showed a tumor of the right adrenal gland with fat density (size: 4 × 3 × 3) (Fig. 1). Surgical abstention and medical supervision for every three months were recommended for this patient. In January 1998, the patient was hospitalized for exacerbation of the symptomatology: loss of weight estimated to ten kilos, nausea and vomiting. The clinical exam showed neither load tangible nor endocrinological signs. The hormonal measure was normal. The CT scan had showed an increase in size of the tumor (8 × 6 × 6 cm). The fat density of this mass evocated a myelolipoma of the right adrenal gland (Fig. 2). A surgical intervention was led by right lombotomy. The exploration revealed a mass of adrenal gland with a yellow color and scattered hemorrhage’s areas. The right kidney was normal. The tumor was
96
A. Benchekroun et al.
Figure 1. Abdominal CT scan (1996). Right adrenal gland tumor (4 cm/3 cm/3 cm) with fatty density.
removed. Macroscopically, the tumor was encapsulated (weight: 300 g, size: 9×7×6 cm). Its color was yellow and at some areas was red. The histological finding showed a proliferation of adipocytes which are dissociated from several hemorrhagic focus and myeloid tissue which contains megakaryocytes, erythroïd cells and lymphocyte. This patient had been kept under observation for three years. He was in good health without pain or abnormality showed by ultrasounography.
3. DISCUSSION In 1905, Girke [3] did the first histological description. In 1929, Oberling [4] suggested the term of myelolipoma (ML). Dyckman and Freedman [5] reported the first operated case in 1957. De Blois and Demay [6] have published the first case of ML which has been diagnosed only by CT scan and final needle aspiration cytology in 1985. Referring to autopsy studies [1, 7], the incidence of this tumor ranges
from 0.01 to 0.2%. Ages of patients varied from 12 to 93 years [10, 11]. The male to female ratio ranged from 2 : 1 to 1 : 1. Usually, the involvement of this tumor is unilateral. Bilateral localization, development in an ectopic adrenal gland or in extra-adrenal gland were reported [7]. Adrenal ML tends to grow very slowly and their size and weight vary significantly. Clinically, the ML is usually symptomless without any function. It is usually discovered incidentally by radiological imaging. Fewer than 40 cases have presented with clinical signs and symptoms of the adrenal mass and complication leading to surgical removal [9]. There have been a few reports of the presence of myelolipomatous tissue in patients with a variety of adrenal function disorders [13]. The most frequent clinical symptoms are abdominal pain and hypertension caused by tumor’s hemorrhage, necrosis and mechanical compression by a bigger tumor [2, 12]. The most important morphologic investigation is CT scan. It allows the detection of adrenal gland’s
97
Adrenal myelolipoma
Figure 2. Abdominal CT scan (1998): fatty load adrenal gland. The size is increased (8 cm/6 cm/6 cm).
tumor whose diameter is bigger than one centimeter with a sensibility higher than 90% [2, 6, 7, 12]. Because of the fat component, it is at the present time, the unique tumor of adrenal gland which can be diagnosed by CT scan only. It appears clearly defined, compressing without invading the adjacent structure, with negative density areas [8, 12]. RMI allows to analyze, in the ML, the non-fat contingents [7]. The main differential diagnosis of a suprarenal fatty mass includes ML, renal angiomyolipoma extending from the upper pole, retroperitoneal lipomas, liposarcoma, teratoma and possible metastasis to adrenal glands from various sources especially lung cancer [9, 12]. In cases when it is impossible to differentiate a ML from an other retroperitoneal tumor, final needle aspiration biopsy guided by ultrasound or ct scan is suggested [9, 12]. The histological diagnosis of ML requires the presence of myeloid and erythroid as well as mature fat cells. Especially the most important cytological feature is the presence of megakaryocytes [6].
Once the diagnosis is certain, the adrenal gland ML doesn’t require any special treatment when it is not symptomatic and the tumor’s diameter measured by ultrasound or CT scan is less than 5 cm. Surgical excision is reserved for all other cases and when cytological and histological examination of the final needle aspiration biopsy material is not sufficient to see fat and myeloid cells together for the definitive diagnosis [8, 12]. Our patient was followed up for three years, the tumor’s size increased and the pain was exacerbate that surgical excision opted for.
4. CONCLUSION The adrenal gland ML is a rare affection. The diagnosis is improved by medical imaging progress. This tumor is usually benign with a good prognosis. The surgical treatment becomes necessary when the tumor’s size increases or it becomes symptomatic.
98
A. Benchekroun et al.
Figure 3. Histology (G × 40). Proliferation of adipocytes myeloid tissue which contains megakaryocytes, erythoïd cells and lymphocytes.
REFERENCES 1 Ishikawa H, et al. Myelolipoma of the adrenal gland. J Urol 1981; 126: 777. 2 Meaglia JP, Smith D. Natural history of an adrenal myelolipoma. J Urol 1992; 147: 1089-90. 3 Girke E. Uber knoken Mark gwebe in der wedermiere. Beitrz Path Anat 1905; 37(Suppl 7): 311. 4 Oberling. Les formations myélolipomateuses. Bull Ass Fr Cancer 1929; 18: 234. 5 Dyckman J, Freedman D. Myelolipoma of the adrenal with clinical features and surgical excision. J Mt Sinai Hosp 1957; 24: 793. 6 Deblois GG, De May RM. Adrenal myelolipoma diagnosis by computed tomography-guided fine-needle aspiration. A case report. Cancer 1985; 55: 848. 7 Daali M, Abbar M, Hajji A. Myélolipome surrénal : une
observation. Presse Méd 1997; 26: 272. 8 Liebman R, Srikantaswomy S. Adrenal myelolipoma demonstrated by computed tomography. J Comp Asst Tomogr 1981; 5: 262. 9 Jongkolnee Settakorn, Chusak Sirivanichai, et al. Fine needle aspiration cytology of adrenal myelolipoma: case report and review of the literature. Diagnostic Cytopathology 1999; 21: 409-12. 10 Scuin F, Gomez P, Martinez I, et al. Angiomyolipoma associated with bilateral adrenal hyperplasia and hypertension. J Urol 1985; 133: 655-7. 11 Plaut A. Myelolipoma in the adrenal cortex. Am J Pathol 1958; 34: 487-515. 12 Wang XYJ, Wu JT, He XG, Pan ZL. CT of adrenal melolipoma report of 7 cases. JBR-BTR 1999; 82: 231-3. 13 Kenney P, Wagner BJ, Rao P, Heffess CS. Melolipoma: CT and pathologic features. Radiology 1998; 208: 87-95.
Surrénales
Adrenal myelolipoma. A case report A. Benchekroun ∗ , H. Jira, M. Ghadouane, E.H. Kasmaoui, M. Zannoud, M. Faik Department of urology A, Ibn Sina hospital Rabat, Morocco
ABSTRACT We report a new case in a 56 years old patient who manifested right side’s pains. The abdominal CT scan showed a tumor of the right adrenal gland with fat density (size: 8 × 6 × 6 cm). This tumor was removed surgically (weight: 300 g). The histological finding revealed a proliferation of adipocyts and myeloid tissue which contains megakaryocytes, erythroid cells and lymphocyts. The outcome is good after three years of surgery. The surgical treatment becomes necessary when the tumor’s size increases or it becomes symptomatic. 2002 Éditions scientifiques et médicales Elsevier SAS adrenal gland / myelolipoma / surgery
RÉSUMÉ Myélolipome de la surrénale. À propos d’un cas. Le myélolipome de la surrénale est une tumeur rare et bénigne. Nous rapportons une nouvelle observation chez un patient âgé de 56 ans qui présentait des douleurs du flanc droit. La tomodensitographie (TDM) abdominale avait montré une tumeur surrénalienne droite de densité graisseuse mesurant 8 × 6 × 6 cm. Une tumeur de 300 g a été enlevée chirurgicalement. L’histologie a montré une prolifération d’adipocytes et un tissu myéloïde contenant des mégacaryocytes, des érythrocytes et des lymphocytes. L’évolution a été bonne avec trois ans de recul. Le traitement chirurgical du myélolipome de la surrénale s’avère nécessaire lorsque celui-ci devient symptomatique ou augmente de taille. 2002 Éditions scientifiques et médicales Elsevier SAS chirurgie / myélolipome / surrénale
(Received 9 April 2001; accepted 4 May 2001)
∗ Correspondence and reprints.
1. INTRODUCTION The myelolipoma (ML) of adrenal gland is a benign and non functionning tumor that is composed of mature adipose tissue and hematopoietic elements similar to those seen in the bone marrows [1, 6, 9]. It’s a rare affection since until 1999, only 300 cases have been reported in world literature [2, 9]. We report another case and call back the characteristics of this pathologic entity.
2. CASE REPORT Mr. B.H., 56 years old, without particular pathology’s history, was examinated in our institution for right side’s pains in 1996. The clinical exam in first was normal. Then the abdominal CT scan showed a tumor of the right adrenal gland with fat density (size: 4 × 3 × 3) (Fig. 1). Surgical abstention and medical supervision for every three months were recommended for this patient. In January 1998, the patient was hospitalized for exacerbation of the symptomatology: loss of weight estimated to ten kilos, nausea and vomiting. The clinical exam showed neither load tangible nor endocrinological signs. The hormonal measure was normal. The CT scan had showed an increase in size of the tumor (8 × 6 × 6 cm). The fat density of this mass evocated a myelolipoma of the right adrenal gland (Fig. 2). A surgical intervention was led by right lombotomy. The exploration revealed a mass of adrenal gland with a yellow color and scattered hemorrhage’s areas. The right kidney was normal. The tumor was
96
A. Benchekroun et al.
Figure 1. Abdominal CT scan (1996). Right adrenal gland tumor (4 cm/3 cm/3 cm) with fatty density.
removed. Macroscopically, the tumor was encapsulated (weight: 300 g, size: 9×7×6 cm). Its color was yellow and at some areas was red. The histological finding showed a proliferation of adipocytes which are dissociated from several hemorrhagic focus and myeloid tissue which contains megakaryocytes, erythroïd cells and lymphocyte. This patient had been kept under observation for three years. He was in good health without pain or abnormality showed by ultrasounography.
3. DISCUSSION In 1905, Girke [3] did the first histological description. In 1929, Oberling [4] suggested the term of myelolipoma (ML). Dyckman and Freedman [5] reported the first operated case in 1957. De Blois and Demay [6] have published the first case of ML which has been diagnosed only by CT scan and final needle aspiration cytology in 1985. Referring to autopsy studies [1, 7], the incidence of this tumor ranges
from 0.01 to 0.2%. Ages of patients varied from 12 to 93 years [10, 11]. The male to female ratio ranged from 2 : 1 to 1 : 1. Usually, the involvement of this tumor is unilateral. Bilateral localization, development in an ectopic adrenal gland or in extra-adrenal gland were reported [7]. Adrenal ML tends to grow very slowly and their size and weight vary significantly. Clinically, the ML is usually symptomless without any function. It is usually discovered incidentally by radiological imaging. Fewer than 40 cases have presented with clinical signs and symptoms of the adrenal mass and complication leading to surgical removal [9]. There have been a few reports of the presence of myelolipomatous tissue in patients with a variety of adrenal function disorders [13]. The most frequent clinical symptoms are abdominal pain and hypertension caused by tumor’s hemorrhage, necrosis and mechanical compression by a bigger tumor [2, 12]. The most important morphologic investigation is CT scan. It allows the detection of adrenal gland’s
97
Adrenal myelolipoma
Figure 2. Abdominal CT scan (1998): fatty load adrenal gland. The size is increased (8 cm/6 cm/6 cm).
tumor whose diameter is bigger than one centimeter with a sensibility higher than 90% [2, 6, 7, 12]. Because of the fat component, it is at the present time, the unique tumor of adrenal gland which can be diagnosed by CT scan only. It appears clearly defined, compressing without invading the adjacent structure, with negative density areas [8, 12]. RMI allows to analyze, in the ML, the non-fat contingents [7]. The main differential diagnosis of a suprarenal fatty mass includes ML, renal angiomyolipoma extending from the upper pole, retroperitoneal lipomas, liposarcoma, teratoma and possible metastasis to adrenal glands from various sources especially lung cancer [9, 12]. In cases when it is impossible to differentiate a ML from an other retroperitoneal tumor, final needle aspiration biopsy guided by ultrasound or ct scan is suggested [9, 12]. The histological diagnosis of ML requires the presence of myeloid and erythroid as well as mature fat cells. Especially the most important cytological feature is the presence of megakaryocytes [6].
Once the diagnosis is certain, the adrenal gland ML doesn’t require any special treatment when it is not symptomatic and the tumor’s diameter measured by ultrasound or CT scan is less than 5 cm. Surgical excision is reserved for all other cases and when cytological and histological examination of the final needle aspiration biopsy material is not sufficient to see fat and myeloid cells together for the definitive diagnosis [8, 12]. Our patient was followed up for three years, the tumor’s size increased and the pain was exacerbate that surgical excision opted for.
4. CONCLUSION The adrenal gland ML is a rare affection. The diagnosis is improved by medical imaging progress. This tumor is usually benign with a good prognosis. The surgical treatment becomes necessary when the tumor’s size increases or it becomes symptomatic.
98
A. Benchekroun et al.
Figure 3. Histology (G × 40). Proliferation of adipocytes myeloid tissue which contains megakaryocytes, erythoïd cells and lymphocytes.
REFERENCES 1 Ishikawa H, et al. Myelolipoma of the adrenal gland. J Urol 1981; 126: 777. 2 Meaglia JP, Smith D. Natural history of an adrenal myelolipoma. J Urol 1992; 147: 1089-90. 3 Girke E. Uber knoken Mark gwebe in der wedermiere. Beitrz Path Anat 1905; 37(Suppl 7): 311. 4 Oberling. Les formations myélolipomateuses. Bull Ass Fr Cancer 1929; 18: 234. 5 Dyckman J, Freedman D. Myelolipoma of the adrenal with clinical features and surgical excision. J Mt Sinai Hosp 1957; 24: 793. 6 Deblois GG, De May RM. Adrenal myelolipoma diagnosis by computed tomography-guided fine-needle aspiration. A case report. Cancer 1985; 55: 848. 7 Daali M, Abbar M, Hajji A. Myélolipome surrénal : une
observation. Presse Méd 1997; 26: 272. 8 Liebman R, Srikantaswomy S. Adrenal myelolipoma demonstrated by computed tomography. J Comp Asst Tomogr 1981; 5: 262. 9 Jongkolnee Settakorn, Chusak Sirivanichai, et al. Fine needle aspiration cytology of adrenal myelolipoma: case report and review of the literature. Diagnostic Cytopathology 1999; 21: 409-12. 10 Scuin F, Gomez P, Martinez I, et al. Angiomyolipoma associated with bilateral adrenal hyperplasia and hypertension. J Urol 1985; 133: 655-7. 11 Plaut A. Myelolipoma in the adrenal cortex. Am J Pathol 1958; 34: 487-515. 12 Wang XYJ, Wu JT, He XG, Pan ZL. CT of adrenal melolipoma report of 7 cases. JBR-BTR 1999; 82: 231-3. 13 Kenney P, Wagner BJ, Rao P, Heffess CS. Melolipoma: CT and pathologic features. Radiology 1998; 208: 87-95.