Subject(s)
Adrenal Cortex Neoplasms/complications , Adrenal Hyperplasia, Congenital/complications , Adrenocortical Carcinoma/complications , Feminization/etiology , Adrenal Cortex Neoplasms/diagnosis , Adrenal Hyperplasia, Congenital/diagnosis , Adrenocortical Carcinoma/diagnosis , Age Factors , Aged , Feminization/diagnosis , Gynecomastia/diagnosis , Gynecomastia/etiology , Humans , MaleABSTRACT
We report the case of a voluminous tumor of the adrenal diagnosed in a young pregnant woman at 26(th) week of amenorrhea. Morphologically, a soft white tumor with haemorragic areas was observed, made of sheets of monomorphous, medium sized, spindle-shaped to polygonal, with high mitotic activity. Tumorous cells expressed cytokeratins AE1/AE3, EMA, and CD99 (expression of vimentin is not relevant). Contemplated diagnoses included poorly differentiated synovialosarcoma, sarcomatoid carcinoma and Ewing tumor. Thanks to molecular biology, showing the specific transcript of Ewing/peripheral primitive neuroectodermal tumor (pPNET) EWS/FLI1, the diagnosis of this atypical tumor in an unusual location was performed. Indeed, 75% of Ewing tumors involve bones (especially, the diaphysis of long bones) and 20 to 25% soft tissues. Primitive visceral involvement is rare; less than 10 cases of adrenal involvement have been reported. The hypothesis that Ewing cell's origin is a mesenchymal stem cell, which may derive from neural crest cell, could explain the uncommon adrenal involvement. Diagnosis of Ewing tumor is based on pathologic and molecular findings, especially in atypical cases.
Subject(s)
Adrenal Gland Neoplasms/pathology , Neuroectodermal Tumors, Primitive/pathology , Pregnancy Complications, Neoplastic/pathology , Sarcoma, Ewing/pathology , Adrenal Gland Neoplasms/chemistry , Adrenal Gland Neoplasms/complications , Adrenal Gland Neoplasms/diagnosis , Adrenal Gland Neoplasms/therapy , Adrenalectomy , Adult , Biomarkers, Tumor/analysis , Carcinoma/diagnosis , Cell Transformation, Neoplastic , Cesarean Section , Chemotherapy, Adjuvant , Combined Modality Therapy , Diagnosis, Differential , Female , Humans , Infant, Newborn , Lymph Node Excision , Mesenchymal Stem Cells/pathology , Neuroectodermal Tumors, Primitive/chemistry , Neuroectodermal Tumors, Primitive/complications , Neuroectodermal Tumors, Primitive/diagnosis , Neuroectodermal Tumors, Primitive/therapy , Oncogene Proteins, Fusion/analysis , Organ Specificity , Pheochromocytoma/diagnosis , Pre-Eclampsia , Pregnancy , Pregnancy Complications, Neoplastic/diagnosis , Pregnancy Complications, Neoplastic/therapy , Proto-Oncogene Protein c-fli-1/analysis , RNA-Binding Protein EWS/analysis , Radiotherapy, Adjuvant , Sarcoma, Ewing/chemistry , Sarcoma, Ewing/complications , Sarcoma, Ewing/diagnosis , Sarcoma, Ewing/therapy , Sarcoma, Synovial/diagnosisABSTRACT
CONTEXT: Effective treatment for the ectopic ACTH secretion syndrome (EAS) remains a therapeutic challenge. Immediate curative surgery of the responsible nonpituitary tumor is often not possible. OBJECTIVE: The objective of the study was to evaluate 1,ortho-1, para'-dichloro-diphenyl-dichloro-ethane (O,p'DDD) therapy in EAS. DESIGN AND PATIENTS: Patients included 36 consecutive patients with EAS from a single center treated between 1990 and 2006. Twenty-three of these patients, including 18 women aged 53.7 +/- 12.9 yr (mean +/- sd), were treated with O,p'DDD. Patient follow-up was 8.04 +/- 9.6 yr. RESULTS: A mean daily O,p'DDD dose of 3.3 +/- 1.2 g Lysodren equivalent was given for a mean duration of 1.8 +/- 2.1 yr. Urinary cortisol decreased from 2603 +/- 3443 microg/d before treatment to 79 +/- 169 microg/d at the time of maximal O,p'DDD efficacy. Urinary cortisol was normalized in 21 of the 23 patients. Adrenal insufficiency was observed in 20 patients. This was associated with clinical improvement of Cushing's syndrome manifestations, including diabetes, hypertension, and hypokalemia. O,p'DDD plasma levels were 10.4 +/- 6.5 microg/ml in the 12 patients tested at the time of adrenal insufficiency. Side effects were observed during the first 6 months in seven of 15 patients (46%). National Cancer Institute-Classification Common Toxicity Criteria grade 1 or 2 digestive or neurologic toxicity resolved after withdrawal or reduction of O,p'DDD. Careful monitoring was essential to long-term control, clinical improvement, and good tolerability. Medical control of the disease allowed the subsequent characterization of tumors in eight of 13 patients with initially occult tumors. CONCLUSION: With close monitoring, O,p'DDD could be a potent medical treatment for long-term control and management of EAS.
