Adrenal Myelolipoma

1
Adrenal Myelolipoma

• Chien-Wen Chou MD
• Endocrinology Metabolism Division
• Chi-Mei Medical Center
• 28 April 2006

2
Case Report (1)

• 44 y/o male with acute hyperglycemia
• B-thalassemia with frequent RBC transfusion
  developed hemachromatosis
• Hepatosplenomegaly due to hemachromatosis with
  hepatitis B
• Bilateral adrenal tumor, left adrenectomy due to
  rupture of myelolipoma
• Hypogonadotropic hypogonadism

3
Case Report (2)

• 880217 MRI right 9 cm, left 12 cm
• 10417 testosterone 23 ng/dL (241-800), FSH 0.3
  uIU/ml(1.4-1.8) LH 0.7 uIU/ml (1.5-9.3)
  metanephrine 107 ug/day (52-341)
• 950419 Echo right 12.8 cm
• 950421 T4 5.05, T3 34.5, TSH 2.69, PRL 9.79,
  Cortisol 8AM 8.17, 4pm 9.42, AC/PC C-peptide
  1.84/1.15 ng/ml, Ferritin gt 2000 ng/ml

4
Abdominal Echo 950420
5
Table of Content

• Introduction
• Age of diagnosis
• Clinical manifestations
• Size and weight
• Functioning or nonfunctioning
• Bilateral
• Extra-adrenal
• Imaging
• Natural history
• Surgical or observation

6
Introduction
7
Introduction

• Myelolipoma may present hematopoietic, fat and
  bone components.
• Myelolipomas of the adrenal gland were first
  described in 1905.
• They are rare cortical, nonfunctioning, benign
  neoplasms, generally unilateral, usually
  discovered by accident or at autopsy.
• Thus they are often classified as
  'incidentaloma'.
• These tumors are more frequent in males 40-60
  years old.
• Most adrenal myelolipomas are small (diameter lt 4
  cm) and asymptomatic (70), but larger tumors may
  cause local symptoms secondary to mechanical
  compression.

8
Age Distribution
9
Adrenal myelolipoma in a child

• The age range of the affected patients is 20-90
  years most are men in their fourth to sixth
  decade.
• A case of adrenal myelolipoma is reported in a
  14-year-old girl.
• Abdominal ultrasound examination revealed a
  left-sided ovarian cyst 13 cm in diameter and an
  8 x 8-cm hyperechoic heterogenous solid mass
  localized in the right adrenal gland.
• The ovarian cyst and adrenal mass were removed
  surgically.
• Histological examination of the adrenal mass
  revealed a proliferation of mature adipose tissue
  with bone marrow-like hematopoietic elements.
• The ovarian cyst was a simple serous cyst.

Pediatr Surg Int. 2005 Jun21(6)500-2. Epub 2005
Apr 5.
10
Size and Weight
11
Giant adrenal myelolipoma report of a case.

• We report the case of a giant adrenal myelolipoma
  in a 51-year old man who presented with a huge
  abdominal mass and abdominal pain.
• The resected tumor weighed 6,000 g and could
  represent the largest such tumor ever documented
  in the literature.

Surg Today. 200434(3)283-5.
12
Functioning
13
Multiple calcified adrenal myelolipoma suggestive
of association of primary aldosteronism report
of a case

• We report a case of adrenal myelolipoma with
  multiple calcification and hypertension.
• A 69-year-old woman visited our hospital with a
  complaint of right flank pain.
• Computed tomography demonstrated a right adrenal
  tumor which was a spherical mass with fat density
  and multiple calcification.
• Adrenal scintigraphy of I-131 adosterol
  demonstrated predominant accumulation of the
  right adrenal gland.
• Selective venous sampling disclosed a high
  aldosterone level (303.7 ng/dl) from the affected
  side. Right adrenalectomy was performed.
• Pathological diagnosis revealed adrenal
  myelolipoma with calcification.
• After the operation the patient became
  normotensive

Hinyokika Kiyo. 1991 Feb37(2)151-5
14
Combined adrenal myelolipoma and medullary
hyperplasia

• A 52-year-old woman with long-standing
  hypertension was evaluated for an incidentally
  discovered large tumor of the left adrenal.
• Left adrenalectomy was performed for a
  presumptive clinical diagnosis of
  pheochromocytoma.
• Histopathologic examination revealed a mixed
  tumor consisting of a large myelolipoma with
  infiltrating foci of adrenal medulla.
• CONCLUSIONS A patient is described with
  hypertension, myelolipoma and adrenal medullary
  hyperplasia following adrenalectomy, however,
  blood pressure and biochemical abnormalities
  normalized

Horm Res. 200462(1)23-6. Epub 2004 May 5.
15
Giant bilateral adrenal myelolipoma associated
with congenital adrenal hyperplasia

• Myelolipomas are mostly clinically inert, only a
  small number of them are associated with
  Cushing's type of endocrine disorders, Conn's
  syndrome, Addison's disease, etc.
• a rare case of a giant bilateral myelolipoma
  emerging out of the adrenal gland cortex in a
  congenital adrenal hyperplasia, with steroid
  21-hydroxylase deficiency, in a woman with
  pronounced virilism.

Zentralbl Chir. 2006 Feb131(1)80-3
16
Adrenal myelolipoma associated with hereditary
spherocytosis

• We report a case of adrenal myelolipoma
  associated with hereditary spherocytosis which
  was treated with splenectomy seventeen years ago.
  
• The hematopoietic stimulus of the hereditary
  spherocytosis might have been associated with the
  development of adrenal myelolipoma in the present
  case.

Int J Urol. 1996 Jan3(1)61-3.
17
Etiology

• Their origin is unclear
• development from rests of mesenchymal stem cells
• embolism of bone marrow
• extramedullary hematopoiesis
• metaplasia of the reticuloendothelial cells of
  blood capillaries.

18
Bilateral
19
Bilateral adrenal myelolipoma a case report

• The patient was a 40-year-old man in whom a
  retroperitoneal tumor was unexpectedly found by
  ultrasonography.
• Computed tomography revealed bilateral adrenal
  masses, 14.0 cm in diameter on the right side,
  and 5.0 cm on the left side, with heterogeneous
  low density components.
• Magnetic resonance imaging also revealed a high
  intensity mass with heterogeneity.
• Laboratory examination of adrenal function
  revealed normal results.
• We confirmed myelolipoma of bilateral adrenal
  gland, and performed right adrenalectomy, and
  left tumor enucleation.
• The right tumor was 14.0 x 12.5 x 10.0 cm in size
  and 920 g in weight, and the left tumor 5.0 x 4.5
  x 4.0 cm in size and 50 g in weight.
• Pathology disclosed an admixture of mature
  adipose tissue and hematopoietic elements
  resembling bone marrow. Histopathological
  diagnosis was adrenal myelolipoma.
• This case was the 6th to be reported in the
  Japanese literature as bilateral adrenal
  myelolipoma.

Hinyokika Kiyo. 1994 Aug40(8)695-8
20
Bilateral synchronous adrenal myelolipomas a
case report

• A 70-year-old man, who was diagnosed by computed
  tomographic scan as having bilateral synchronous
  adrenal myelolipomas 6 years ago during the
  follow-up of fatty liver, underwent tumor
  resection at our Department because of growth of
  bilateral tumors without any subjective symptoms.
  
• Histopathological examination revealed mature
  adipose tissue cells without atypism and areas of
  hematopoietic tissue, including the myelotic,
  lymphotic, erythrocytic, and megakaryocytic
  cells.
• The diagnosis of myelolipoma was confirmed.
• The in vivo doubling time of bilateral tumors was
  16.1 months and 31.3 months, respectively

Hinyokika Kiyo. 1998 Nov44(11)801-3.
21
Bilateral giant adrenal myelolipomas a case
report

• We report a case of bilateral giant adrenal
  myelolipoma producing abdominal pain in a
  54-year-old man, who presented to his primary
  care physician with complaints of right shoulder
  pain with vague abdominal discomfort.
• Imaging studies identified bilateral suprarenal
  masses measuring 12 x 14 cm on the right and 8 x
  10 cm on the left.
• These masses were consistent with fatty tissue
  radiographically. In addition a focal 5 x 4-cm
  mass was identified in the transverse colon.
• Because the patient was symptomatic and a
  diagnosis of liposarcoma could not be excluded he
  was taken to the operating room for exploratory
  laparotomy with excision of the masses and a
  transverse colectomy.
• Final histologic analysis identified bilateral
  adrenal myelolipomas and a solitary lipoma of the
  transverse colon.
• His postoperative course was uneventful with
  relief of the pain.

• Am Surg. 2002 Jun68(6)588-9.

22
Extra-Adrenal
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Extra-adrenal myelolipoma report of two cases.

• several extra-adrenal myelolipomas (EAMLs) have
  been reported.
• The typical EAML is a solitary, well-defined mass
  within the abdomen, most commonly in the
  retroperitoneal presacral area.
• EAMLs may produce symptoms related to their mass
  effect, but they are occasionally incidental
  findings.
• Most commonly, the patient is older than 40 years
  and has no hematologic abnormalities.
• It is important to distinguish EAMLs from other
  soft tissue tumors, in particular liposarcomas,
  myxoid malignant fibrous histiocytomas, and
  extramedullary hematopoietic tumors.
• We discuss two cases of EAML. The first was in
  the retroperitoneum of a 76-year-old woman. It is
  the largest EAML ever reported, measuring 26 cm x
  15 cm x 11 cm.
• The second, a presacral mass in a 68-year-old
  man, was diagnosed preoperatively by percutaneous
  computed tomography-guided fine needle aspiration
  biopsy.

South Med J. 1995 Jun88(6)639-43
24
Bilateral thoracic extraadrenal myelolipoma.

• Extraadrenal myelolipoma may occur in the
  retroperitoneum, stomach, liver, lung, and in 3
  of cases even in the mediastinum.
• We present a 65-year-old female patient with
  unspecific clinical symptoms.
• Routine chest roentgenograms revealed bilateral
  widening of the posterior mediastinum.
• Computed tomography showed bilateral,
  paravertebral lesions of 4.5 and 6.5 cm in
  diameter, respectively.
• After surgical removal, bilateral thoracic
  myelolipoma was pathomorphologically diagnosed.
• The imaging differential diagnosis of bilateral
  solid lesions in the posterior mediastinum
  including lymph node metastases, lymphomas,
  neurogenic tumors and extramedullary
  hematopoietic tumors is discussed.

Eur J Cardiothorac Surg. 2004 Dec26(6)1220-2
25
Myelolipoma CT and pathologic features

• All cases of myelolipoma accessioned by the Armed
  Forces Institute of Pathology from 1981 through
  1997 were reviewed. CT images were scored for the
  location and size of each myelolipoma and the
  presence of calcification, hemorrhage, fat, and
  pseudocapsule.
• Pathologic findings for the pattern of fat and
  bone marrow elements were correlated with CT
  findings.
• RESULTS In 74 patients, 86 myelolipomas were
  found, of which 72 were in an adrenal gland
  (eight were bilateral), and 14 extra-adrenal
  masses were found in 10 patients.
• Four clinicopathologic patterns emerged (a)
  isolated adrenal myelolipoma in 37 patients (fat
  evident at CT, no other disorders present) (b)
  myelolipoma with hemorrhage in nine patients
  (imaging features similar to those of isolated
  adrenal myelolipomas but larger mean diameter,
  14.2 vs 9.9 cm P .01 (c) extra-adrenal
  myelolipoma in 10 patients (imaging findings
  similar to those of adrenal myelolipomas, found
  most often in the retroperitoneum) and (d)
  myelolipomatous foci within other adrenal
  pathologic conditions in 18 patients (smaller,
  lower fat content, more heavily calcified).
• CONCLUSION Myelolipomas are adrenal or
  extra-adrenal masses, with hemorrhage more common
  in larger lesions (diameter, gt 10 cm). The CT
  appearance of myelolipomatous foci, which can be
  found within other pathologic adrenal conditions,
  is different from that of isolated adrenal
  myelolipomas.

Radiology. 1998 Jul208(1)87-95.
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Pathology AML with hemorrhage
29
The natural history and treatment of adrenal
myelolipoma

• A retrospective review of medical records and
  radiographic imaging studies of 20 patients
  diagnosed with adrenal myelolipoma was performed.
  
• RESULTS Of 20 patients 4 presented with
  abdominal pain and 1 had Cushing's syndrome. The
  remaining tumors were discovered incidentally.
• Four patients underwent surgery because of
  abdominal pain in 2, adrenal hyperfunction
  (Cushing's syndrome) in 1 and a tumor 10.5 cm. in
  largest dimension in 1.
• Of 15 patients (16 adrenal myelolipomas) followed
  without surgical intervention for an average of
  3.2 years (range 0.3 to 10.8) 13 remained
  asymptomatic and 2 experienced persistent, vague
  abdominal discomfort. One patient was lost to
  followup.
• A total of 13 tumors from 12 patients was
  serially imaged, with tumor size increasing in 6,
  decreasing in 2 and remaining unchanged in 5.
• CONCLUSIONS These data suggest that the majority
  of adrenal myelolipomas can be treated
  conservatively. While tumors can become enlarged,
  they also exhibit variable growth, and size and
  growth rate do not necessarily correlate with
  symptoms. Computerized tomography can be used for
  diagnosis.

J Urol. 1997 Apr157(4)1213-6.
30
Managment

• Asymptomatic small tumors, smaller than 4 cm, may
  be monitored with watchful waiting.
• Symptomatic tumors greater than 4 cm should be
  extirpated because of the risk of spontaneous
  rupture with retroperitoneal bleeding.
• Bilateral adrenalectomy for big tumors implies
  medical replacement with hydrocortisone.

31
Embolization for spontaneous retroperitoneal
hemorrhage from adrenal myelolipoma.

• To our knowledge, there have been only 11
  reported cases of ruptured adrenal myelolipoma in
  the literature.
• We report a 37-year-old female patient who
  underwent transcatheter arterial embolization
  using gelatin sponge particles for
  retroperitoneal hemorrhage from adrenal
  myelolipoma, 8x6x6 cm in size.
• This case, which is the second in the literature,
  illustrates the usefulness of embolization to
  achieve hemostasis prior to subsequent tumor
  resection.

Radiat Med. 2003 Sep-Oct21(5)214-9
32
Adrenal myelolipoma--report of two cases

• We report two cases of adrenal myelolipoma.
• One was a middle-aged woman with right flank
  pain. Tumor size increased 8 years later.
• The other patient was a 63-year-old man
  presenting with right flank soreness.
• The right adrenal tumor was found by abdominal
  sonography.
• Both of them received adrenalectomy to relieve
  symptoms and the pathologic results showed
  adrenal myelolipoma.

Kaohsiung J Med Sci. 2000 Oct16(10)542-7.