Giant Bilateral Adrenal Myelolipomas in a Non-Compliant Patient with Congenital Adrenal Hyperplasia.

BACKGROUND 21-hydroxylase deficiency, an essential enzyme for glucocorticoid and mineralocorticoid synthesis, is the cause of congenital adrenal hyperplasia (CAH) in more than 95% of cases. It is an autosomal recessive disorder encoded by the CYP21A2 gene, categorized into classical forms, which encompass the salt-wasting (SW) and simple virilizing (SV) forms, as well as the nonclassical form (NC). The aim of medical treatment is to replace missing glucocorticoids and, if necessary, mineralocorticoids, while also reducing elevated adrenal androgens. CASE REPORT We present the case of a 42-year-old woman with CAH who discontinued therapy during adolescence and was admitted to hospital with fatigue, nausea, and severe abdominal pain. A CT scan showed an extreme enlargement of the adrenal glands. Laboratory tests revealed elevated levels of 17-hydroxyprogesterone and other adrenal androgens, along with normal plasma metanephrine levels. Decreased morning cortisol levels suggested partial adrenal insufficiency requiring glucocorticoid replacement therapy. Due to the development of several serious complications and clinical deterioration, the multidisciplinary team recommended bilateral removal of masses measuring 300×250×200 mm on the right side and 250×200×200 mm on the left side. Histological and immunochemical examination confirmed the presence of giant myelolipomas with adrenal cortex hyperplasia. CONCLUSIONS Adrenal tumors, particularly myelolipomas, have a higher prevalence in patients with CAH. Our case report provides further evidence of the suspected link between non-compliant CAH therapy and the development of myelolipomas, along with promotion of their pronounced growth.

Simultaneous adrenal and retroperitoneal myelolipoma resected by laparoscopic surgery: a challenging case.

BACKGROUND: Myelolipoma is a benign neoplasm of the adrenal cortex, composed of fat and hematopoietic cells. Although myelolipoma is benign, differentiation from adrenocortical cancer may be difficult. The presence of adrenal and extra-adrenal myelolipomas simultaneously is sporadic, making it a challenging case, especially when the preoperative diagnosis is ambiguous. CASE PRESENTATION: A 65-year-old man was referred to our clinic due to a mass in the adrenal fossa. In the abdominopelvic computed tomography (CT), a well-circumscribed fat-containing 78 × 61 × 65 mm bi-lobulated mass was reported in the left adrenal fossa. The first differential diagnosis was myelolipoma. The patient was then referred to our clinic for a mass excision. He was asymptomatic and was scheduled to undergo laparoscopic-assisted adrenalectomy. After adrenalectomy and mass dissection, surprisingly, another mass was detected in the retroperitoneal area. The second mass was also dissected. The final diagnosis was myelolipoma for both masses. The patient has been symptom-free for nine months after the operation. CONCLUSION: Simultaneous adrenal and extra-adrenal myelolipoma should be considered as one of the differential diagnoses. However, because this situation is extremely rare, the probability of malignancy should be highly regarded, and we suggest an obsessive approach when approaching this condition. It is essential to manage these cases on a case-by-case basis and tailor the management concerning intraoperative biopsy, the intraoperative appearance of tumors, and the location of extra-adrenal masses.

Spinal myelolipoma - an extremely rare pathology within the lumbar spine: a case report and literature review.

Myelolipoma is a benign tumor containing mature adipose cells and a combination of myeloid and erythroid elements. This tumor is typically found in the adrenal glands; however, it has been detected outside the adrenal glands in rare cases. We report an extremely rare case of myelolipoma in the lumbar spine causing significant neural compression due to the involvement of the posterior spinal elements. Given the significant neurological deficit, the patient was surgically managed as soon as possible. Extra-adrenal myelolipomas are rare lesions, and only one case has been reported in the spine so far. However, this diagnosis should be considered in cases with its characteristic imaging features.

Giant adrenal myelolipoma in a young male double heterozygous for HbS and beta-thalassemia trait, clinically simulating retroperitoneal sarcoma.

ABSTRACT: A 57-year-old male had abdominal pain and distension for 6-7 months with a palpable swelling in the right lumbar region. Contrast-enhanced computed tomography abdomen showed a large heterogeneous lesion with fat density measuring 22 cm ´ 16.5 cm in the right suprarenal region. Laparotomy was done which showed an encapsulated mass measuring 21 cm ´ 14 cm ´ 5 cm. Cut section revealed yellowish areas admixed with hemorrhage and large areas of necrosis. Microscopy revealed adrenal myelolipoma. The patient was found to have sickling positive. High-performance liquid chromatography showed double heterozygous for HbS and beta-thalassemia trait. The association of giant adrenal myelolipoma with double heterozygous for HbS and beta-thalassemia trait is rare, and as clinically it simulates retroperitoneal sarcoma, awareness of this rare entity is critical for its accurate diagnosis and proper management.

[Primary Myelolipoma of the Posterior Mediastinum:Report of a Case].

The patient was a 66-year-old male. Computed tomography( CT) scan revealed a tumor of the posterior mediastinum 2 years before and grew slowly 49.0 to 51.4 mm in the longest diameter of the coronal slice. Thoracoscopic surgery was performed to resect it. The pathological examination revealed a predominantly mature adipose tissue with hematopoietic tissue in hematoxylin and eosin( HE) staining and the hematopoietic tissue was comprised of mature erythroblasts, megakaryocytes, and granulocytes in immune staining, which diagnosed it as myelolipoma. Incidence of myelolipoma which originates from besides adrenal glands is reported 0.08 to 0.2%. As far as we can search, 40 cases of myelolipoma of the posterior mediastinum are in the literatures.

Bilateral giant adrenal myelolipoma: A rare scenario.

Adrenal myelolipoma is a benign tumor-like growth, composed of mature fat cells and bone marrow elements. We report a case of a 44-year lady who presented with a complaint of pain in the abdomen. The only positive finding was contrast-enhanced computed tomography (CECT) whole abdomen, which was suggestive of heterogeneously enhancing hypodense lesion of size 130 mm × 105 mm with few calcifications and 103 mm × 75 mm with intralesional fat attenuation in right and left adrenals. Rest laboratory parameters were normal. Only a few cases so far have been reported for bilateral adrenal myelolipoma but what stands out in our case is its giant size and bilaterality, managed surgically without any complications.

[Adrenal myelolipoma, review of the literature in Mexico apropos of two cases]. / Mielolipoma Suprarrenal, revisión de literatura en México a propósito de dos casos.

Background: Adrenal myelolipomas (ML) are rare benign neoplasms compound of adipose and myeloid tissue. Clinically they are usually asymptomatic, being diagnosed generally by incident. In Mexico, there are only 32 published cases of ML, these occur between 37 and 65 years, with the male-female ratio being 1:1.1, clinically they present with abdominal or lumbar pain, open surgery being the main surgical approach (89%). Clinical case: We made a literature review of ML in Mexico and present two clinical cases: a 67-year-old man in followup for diverticular disease and a 40-year-old woman with pain in the left upper quadrant. In both cases, tumor resection was performed measuring 9.5 cm and 13.3 cm long respectively. Conclusions: We present two new cases in our country that correspond to incidentalomas. In both cases, surgery was performed to confirm the diagnosis, as well as to prevent possible complications.

Introducción: los mielolipomas suprarrenales (ML) son neoplasias benignas poco frecuentes constituidas por tejido adiposo y mieloide. Clínicamente asintomáticas, suelen ser diagnosticados incidentalmente. En México existen solo 32 casos publicados de ML, presentándose en pacientes de entre 37 a 65 años, siendo la relación hombre-mujer 1:1.1, el síntoma más comúnmente reportado es dolor abdominal inespecífico, y la cirugía abierta es el principal abordaje quirúrgico empleado en nuestro país (89%). Caso clínico: presentamos una recopilación de la literatura actual sobre ML en México, además de dos casos clínicos de pacientes con ML: un hombre de 67 años con enfermedad diverticular y una mujer de 40 años con dolor en hipocondrio; en ambos se realizó resección tumoral, midiendo 9.5 cm y 13.3 cm, respectivamente. Conclusiones: presentamos dos casos nuevos en nuestro país que corresponden a incidentalomas. En ambos casos la cirugía se realizó para confirmar el diagnóstico, así como para prevenir posibles complicaciones.

Anterior Mediastinal Myelolipoma: A Case Report and Review of the Literature.

BACKGROUND Myelolipoma is a benign tumor, commonly found in the supra-renal gland, which is composed of mature fatty tissue admixed with hematopoietic elements. However, there are several extra-adrenal sites reported in the literature and thoracic myelolipoma is an unusual location for extra-adrenal myelolipoma. CASE REPORT We present the case of a 71-year-old man previously diagnosed with hypertension who was admitted due to a motor vehicle injury with a lumbar spine fracture. The patient developed non-ST segment elevation myocardial infarction during admission. A coronary angiogram revealed three-vessel disease. Triple coronary artery bypass grafting (CABG) surgery was planned. Upon opening the chest through median sternotomy, a retrosternal adherent mass was incidentally discovered. The mass was excised and histopathological evaluation showed it was myelolipoma in the anterior part of the mediastinum. CONCLUSIONS It is well known that myelolipoma occurs in extra-adrenal sites, and is rarely found at unexpected site, as in our case, which was found incidentally at the anterior mediastinum. With an extensive literature review, we found only 1 case located in the anterior mediastinum. It is crucial to know that myelolipoma can occur in the anterior mediastinum to avoid pitfalls with other differential diagnoses, especially when it is found incidentally and requires a frozen section examination, as it is difficult to diagnose through radiologic imaging only because it can overlap with tumors that are rich in either adipose tissue or hematopoietic elements. However, it affects patient management, and patients usually need only follow-up instead of going through invasive procedures for resection of non-functional tumors, especially in older patients or patients with comorbid diseases.

Mielolipoma suprarrenal, revisión de literatura en México a propósito de dos casos / Adrenal myelolipoma, review of the literature in Mexico apropos of two cases

Introducción: los mielolipomas suprarrenales (ML) son neoplasias benignas poco frecuentes constituidas por tejido adiposo y mieloide. Clínicamente asintomáticas, suelen ser diagnosticados incidentalmente. En México existen solo 32 casos publicados de ML, presentándose en pacientes de entre 37 a 65 años, siendo la relación hombre-mujer 1:1.1, el síntoma más comúnmente reportado es dolor abdominal inespecífico, y la cirugía abierta es el principal abordaje quirúrgico empleado en nuestro país (89%). Caso clínico: presentamos una recopilación de la literatura actual sobre ML en México, además de dos casos clínicos de pacientes con ML: un hombre de 67 años con enfermedad diverticular y una mujer de 40 años con dolor en hipocondrio; en ambos se realizó resección tumoral, midiendo 9.5 cm y 13.3 cm, respectivamente. Conclusiones: presentamos dos casos nuevos en nuestro país que corresponden a incidentalomas. En ambos casos la cirugía se realizó para confirmar el diagnóstico, así como para prevenir posibles complicaciones.

Background: Adrenal myelolipomas (ML) are rare benign neoplasms compound of adipose and myeloid tissue. Clinically they are usually asymptomatic, being diagnosed generally by incident. In Mexico, there are only 32 published cases of ML, these occur between 37 and 65 years, with the male-female ratio being 1:1.1, clinically they present with abdominal or lumbar pain, open surgery being the main surgical approach (89%). Clinical case: We made a literature review of ML in Mexico and present two clinical cases: a 67-year-old man in followup for diverticular disease and a 40-year-old woman with pain in the left upper quadrant. In both cases, tumor resection was performed measuring 9.5 cm and 13.3 cm long respectively. Conclusions: We present two new cases in our country that correspond to incidentalomas. In both cases, surgery was performed to confirm the diagnosis, as well as to prevent possible complications

Epidural myelolipoma in a Silken Windhound.

A 12-year-old female spayed, Silken Windhound dog was presented with a 3-month history of lethargy and cervical and lumbosacral spinal pain. No significant abnormalities were noted on CBC or serum biochemical assays. Magnetic resonance imaging of the spine demonstrated a soft tissue mass within the ventral and right epidural space at the level of the L7 vertebra. During surgery, a pale brown mass was identified within the epidural fat. Cytologic and histopathologic examinations demonstrated that the mass was composed of adipose tissue and hematopoietic elements, consistent with a myelolipoma. The lumbosacral spinal pain resolved after surgery. Epidural myelolipomas are rarely reported in the human and veterinary literature.

Nontraumatic hemoabdomen and pancytopenia secondary to myelolipoma in a cat.

OBJECTIVE: (1) To report an unusual etiology for nontraumatic hemoabdomen in cats, and (2) to describe onset and recovery from severe, unexpected pancytopenia seen after surgical removal of a large intra-abdominal myelolipoma. CASE SUMMARY: A 14-year-old neutered male domestic shorthair cat was presented for emergent treatment of suspected nontraumatic hemoabdomen. A hyperechoic mass, with ultrasonographic echogenicity similar to fat, was found in the right cranial abdomen and believed to be associated with the mesentery. Cytological examination of abdominal fluid identified marked extramedullary hematopoiesis within the hemorrhagic effusion. Exploratory laparotomy identified a hepatic mass, which was resected, and revealed to be a hepatic myelolipoma on histopathological examination. The patient's initial recovery was uneventful. However, continued hyporexia resulted in readmission 4 days postoperatively, at which time the patient was found to have a profound, tri-lineage pancytopenia, and cytological evidence indicative of bone marrow recovery. The pancytopenia resolved with continued medical management and supportive care. NEW OR UNIQUE INFORMATION PROVIDED: Ruptured myelolipoma is not a commonly considered differential for nontraumatic hemoabdomen in cats. Furthermore, severe pancytopenia is unexpected following surgical resection of a myelolipoma. This case provides a unique clinical presentation of both nontraumatic hemoabdomen and bone marrow recovery.

Unique case of metachronous adrenal and hepatic myelolipomas.

We present a unique case of metachronous adrenal and hepatic myelolipomas in a 62-year-old man. Myelolipomas are most commonly found in the adrenal gland, with extra-adrenal sites such as the lung, retroperitoneum and liver reported. They are a form of benign mesenchymal tumour, with only 25 case reports of a hepatic location published to date. The are no guidelines to its management. Our patient underwent resection due to pain, and after a complicated postoperative period is pain free.

MYELOLIPOMA COEXISTENCE WITH GLUCOCORTICOID AND ANDROGEN SECRETING ADRENOCORTICAL CARCINOMA: SLOW AND BENIGN CLINICAL COURSE.

We present a case of androgen and glucocorticoid secreting adrenocortical carcinoma with concomitant myelolipoma. A giant adrenal tumor which was initially nonfunctional was reassessed four years later due to the patient's refusal to treat. The patient was a 48-year-old woman with hypertension and acne lesions on the face. Laboratory findings were consistent with glucocorticoid and androgen hypersecretion. Computed tomography revealed a heterogeneously contrasting mass of 145x118x100 mm with lobular contour and soft tissue areas. The patient underwent left laparoscopic transperitoneal adrenalectomy with three port technique. There were no complications in the perioperative period. The resected specimen weighed 850 grams. Pathological findings showed a combination of myelolipoma-adrenal cortical cancer. In the postoperative period, hypertension improved and the hormone panel was normalized. Postoperative computed tomography and PET-CT showed no residual mass and metastasis. Although imaging is compatible with benign masses such as myelolipoma, coexistence of ACC-myelolipoma should be kept in mind and functional evaluation should be performed.