Correspondence
Primitive neuroectodermal tumor of adrenal: Clinical presentation and outcomes ABSTRACT Primitive neuroectodermal tumor (PNET) of adrenal is an extremely rare tumor of neural crest origin. A nonfunctional left adrenal mass (14.6 × 10.5 × 10.0 cm) on computed tomography (CT) was detected in a 40-year-old lady with abdominal pain, swelling, and left pleural effusion. She underwent left adrenalectomy and left nephrectomy with retroperitoneal resection. Histopathology revealed sheets and nest of oval tumor cells with hyperchromatic nuclei, prominent nucleoli, scanty cytoplasm, brisk mitotic activity, necrosis, lymphovascular invasion, capsular invasion, and extension to the surrounding muscles; staining positive for Mic-2 (CD-99 antigen), vimentin, synaptophysin, and Melan-A. Thoracocentesis, pleural fluid study, and pleural biopsy did not show metastasis. She responded well to vincristine, adriamycin, and cyclophosphamide followed by ifosfamide and etoposide (IE). This is the first report of adrenal peripheral PNET (pPNET) from India. This report intends to highlight that pPNET should be suspected in a patient presenting with huge nonfunctional adrenal mass which may be confused with adrenocortical carcinoma. KEY WORDS: Adrenal, Ewing’s tumor, incidentaloma, primitive neuroectodermal tumor
INTRODUCTION Primitive neuroectodermal tumor (PNET) is an extremely rare malignant tumor with poor prognosis, of neural crest origin, arising mainly from the central nervous system (cPNET) or rarely from the peripheral tissue (pPNET).[1] pPNET has been reported most commonly from the soft tissues of extremities, chest wall, paravertebral, or retroperitoneal areas and constitutes 1% of all sarcomas.[2] Even rarely PNETs have been reported from solid organs, the most common being kidney,[3-5] others being ovary, vagina, testis, uterus, cervix uteri, urinary bladder, parotid gland, heart, lung, rectum, pancreas, liver, and gall bladder.[6] pPNET from adrenals is extremely rare with few isolated reports.[7-11] We report a lady presenting with abdominal mass and left pleural effusion diagnosed to have PNET of left adrenal. CASE REPORT A 40-year-old lady presented with abdominal pain and swelling for 4 weeks and respiratory distress of 2 weeks duration. Examination was significant for tachycardia, tachypnea, left-sided pleural effusion, and an abdominal lump palpable in the left lumbar region. Ultrasonography abdomen revealed left adrenal mass. She did not have any stigmata of Cushing’s syndrome. Blood pressure was normal. Computerized tomography (CT) abdomen for better characterization of the abdominal mass revealed
14.6 × 10.5 × 10.0 cm heterogeneous left adrenal mass with areas of necrosis [Figure 1a and b]. Chest X-ray (CXR) confirmed large left-sided pleural effusion. Investigations were significant for normal morning cortisol, normal overnight dexamethasone suppression test, and normal urinary metanephrines [Table 1]. Thoracocentesis with pleural biopsy was done. Biochemistry and cytology of pleural fluid was suggestive of exudative effusion [Table 1]. Study for malignant cells in the pleural fluid was negative. Pleural biospy did not reveal any evidence of tuberculosis or malignancy. Repeat CXR about a month after the initial CXR showed a decrease in pleural effusion with presence of iatrogenic hydropneumothorax. She underwent explorative laprotomy with removal of left adrenal mass (15 × 13 × 10 cm), left kidney (10 × 5 × 4 cm), and a section of the retroperitoneal tissue as the mass was infiltrating the retroperitoneal muscles. Histopathology revealed sheets and nest (rosettes) of oval tumor cells with hyperchromatic nuclei and scanty cytoplasm in a fibrous stroma [Figure 2] with brisk mitotic activity, extensive areas of necrosis [Figure 3], lymphovascular invasion [Figure 4a and b], capsular invasion and extension to the surrounding muscles. Perineural invasion and invasion into the kidney was not demonstrated. Immunohistochemistry revealed tumor cells expressing Mic-2 (CD-99 antigen), vimentin, synaptophysin, and Melan-A (focally and weakly); but negative for cytokeratin, EMA, CK-8/18 chromogranin A, and calretinin. Hence a diagnosis of primitive neuroectodermal tumor of left adrenal
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
Deep Dutta, K. S. Shivaprasad, Ram Narayan Das1, Sujoy Ghosh, Subhankar Chowdhury Departments of Endocrinology and Metabolism and 1 Pathology, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, India For correspondence: Dr. Deep Dutta, Room-9A, 4th Floor Ronald Ross Building, Department of Endocrinology and Metabolism, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, 244 AJC Bose Road, Kolkata - 700 020, India. E-mail: deepdutta2000@ yahoo.com
Access this article online Website: www.cancerjournal.net DOI: 10.4103/0973-1482.126459 PMID: 24518722 Quick Response Code:
709
Dutta, et al.: PNET of adrenal
a
b
Figure 1: (a) Computerized tomography (CT) of the abdomen showing heterogeneous left adrenal mass with areas of necrosis (black arrow), (b) Scannogram of the abdomen showing huge left adrenal mass (black arrow) compressing the spleen superior to it along with left pleural effusion Figure 2: Hematoxylin and eosin staining of the tumor showing small round blue tumor cells with prominent nuclei and nucleoli, scant cytoplasm, arranged in nests (rosettes) in the background of fibrous stroma
a
b
Figure 4: (a) Tumor cells seen infiltrating into the blood vessel. (b) Tumor cells seen in the blood vessel distant from the primary tumor (low magnification) Figure 3: Small round blue tumor cells seen with adjacent area of necrosis (black arrow)
Table 1: Baseline hormonal, biochemical, and pleural fluid parameters Hormonal and biochemical study 8.00 am Cortisol (mcg/dl) (6-32) ONDST cortisol (mcg/dl) (
Primitive neuroectodermal tumor of adrenal: Clinical presentation and outcomes ABSTRACT Primitive neuroectodermal tumor (PNET) of adrenal is an extremely rare tumor of neural crest origin. A nonfunctional left adrenal mass (14.6 × 10.5 × 10.0 cm) on computed tomography (CT) was detected in a 40-year-old lady with abdominal pain, swelling, and left pleural effusion. She underwent left adrenalectomy and left nephrectomy with retroperitoneal resection. Histopathology revealed sheets and nest of oval tumor cells with hyperchromatic nuclei, prominent nucleoli, scanty cytoplasm, brisk mitotic activity, necrosis, lymphovascular invasion, capsular invasion, and extension to the surrounding muscles; staining positive for Mic-2 (CD-99 antigen), vimentin, synaptophysin, and Melan-A. Thoracocentesis, pleural fluid study, and pleural biopsy did not show metastasis. She responded well to vincristine, adriamycin, and cyclophosphamide followed by ifosfamide and etoposide (IE). This is the first report of adrenal peripheral PNET (pPNET) from India. This report intends to highlight that pPNET should be suspected in a patient presenting with huge nonfunctional adrenal mass which may be confused with adrenocortical carcinoma. KEY WORDS: Adrenal, Ewing’s tumor, incidentaloma, primitive neuroectodermal tumor
INTRODUCTION Primitive neuroectodermal tumor (PNET) is an extremely rare malignant tumor with poor prognosis, of neural crest origin, arising mainly from the central nervous system (cPNET) or rarely from the peripheral tissue (pPNET).[1] pPNET has been reported most commonly from the soft tissues of extremities, chest wall, paravertebral, or retroperitoneal areas and constitutes 1% of all sarcomas.[2] Even rarely PNETs have been reported from solid organs, the most common being kidney,[3-5] others being ovary, vagina, testis, uterus, cervix uteri, urinary bladder, parotid gland, heart, lung, rectum, pancreas, liver, and gall bladder.[6] pPNET from adrenals is extremely rare with few isolated reports.[7-11] We report a lady presenting with abdominal mass and left pleural effusion diagnosed to have PNET of left adrenal. CASE REPORT A 40-year-old lady presented with abdominal pain and swelling for 4 weeks and respiratory distress of 2 weeks duration. Examination was significant for tachycardia, tachypnea, left-sided pleural effusion, and an abdominal lump palpable in the left lumbar region. Ultrasonography abdomen revealed left adrenal mass. She did not have any stigmata of Cushing’s syndrome. Blood pressure was normal. Computerized tomography (CT) abdomen for better characterization of the abdominal mass revealed
14.6 × 10.5 × 10.0 cm heterogeneous left adrenal mass with areas of necrosis [Figure 1a and b]. Chest X-ray (CXR) confirmed large left-sided pleural effusion. Investigations were significant for normal morning cortisol, normal overnight dexamethasone suppression test, and normal urinary metanephrines [Table 1]. Thoracocentesis with pleural biopsy was done. Biochemistry and cytology of pleural fluid was suggestive of exudative effusion [Table 1]. Study for malignant cells in the pleural fluid was negative. Pleural biospy did not reveal any evidence of tuberculosis or malignancy. Repeat CXR about a month after the initial CXR showed a decrease in pleural effusion with presence of iatrogenic hydropneumothorax. She underwent explorative laprotomy with removal of left adrenal mass (15 × 13 × 10 cm), left kidney (10 × 5 × 4 cm), and a section of the retroperitoneal tissue as the mass was infiltrating the retroperitoneal muscles. Histopathology revealed sheets and nest (rosettes) of oval tumor cells with hyperchromatic nuclei and scanty cytoplasm in a fibrous stroma [Figure 2] with brisk mitotic activity, extensive areas of necrosis [Figure 3], lymphovascular invasion [Figure 4a and b], capsular invasion and extension to the surrounding muscles. Perineural invasion and invasion into the kidney was not demonstrated. Immunohistochemistry revealed tumor cells expressing Mic-2 (CD-99 antigen), vimentin, synaptophysin, and Melan-A (focally and weakly); but negative for cytokeratin, EMA, CK-8/18 chromogranin A, and calretinin. Hence a diagnosis of primitive neuroectodermal tumor of left adrenal
Journal of Cancer Research and Therapeutics - October-December 2013 - Volume 9 - Issue 4
Deep Dutta, K. S. Shivaprasad, Ram Narayan Das1, Sujoy Ghosh, Subhankar Chowdhury Departments of Endocrinology and Metabolism and 1 Pathology, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, India For correspondence: Dr. Deep Dutta, Room-9A, 4th Floor Ronald Ross Building, Department of Endocrinology and Metabolism, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, 244 AJC Bose Road, Kolkata - 700 020, India. E-mail: deepdutta2000@ yahoo.com
Access this article online Website: www.cancerjournal.net DOI: 10.4103/0973-1482.126459 PMID: 24518722 Quick Response Code:
709
Dutta, et al.: PNET of adrenal
a
b
Figure 1: (a) Computerized tomography (CT) of the abdomen showing heterogeneous left adrenal mass with areas of necrosis (black arrow), (b) Scannogram of the abdomen showing huge left adrenal mass (black arrow) compressing the spleen superior to it along with left pleural effusion Figure 2: Hematoxylin and eosin staining of the tumor showing small round blue tumor cells with prominent nuclei and nucleoli, scant cytoplasm, arranged in nests (rosettes) in the background of fibrous stroma
a
b
Figure 4: (a) Tumor cells seen infiltrating into the blood vessel. (b) Tumor cells seen in the blood vessel distant from the primary tumor (low magnification) Figure 3: Small round blue tumor cells seen with adjacent area of necrosis (black arrow)
Table 1: Baseline hormonal, biochemical, and pleural fluid parameters Hormonal and biochemical study 8.00 am Cortisol (mcg/dl) (6-32) ONDST cortisol (mcg/dl) (
