Melanotic schwannoma of adrenal gland - A rare entity/ diagnostic dilemma
 

Melanotic schwannoma of adrenal gland - A rare entity/ diagnostic dilemma

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Melanotic schwannoma of adrenal gland is very rare entity with

Melanotic schwannoma of adrenal gland is very rare entity with
only two series and less than 100 cases reported in literature. We report a case of melanotic
schwannoma.

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    Melanotic schwannoma of adrenal gland - A rare entity/ diagnostic dilemma Melanotic schwannoma of adrenal gland - A rare entity/ diagnostic dilemma Document Transcript

    • Melanotic schwannoma of adrenal gland - A rare entity/ diagnostic dilemma
    • Case Report Melanotic schwannoma of adrenal gland e A rare entity/ diagnostic dilemma Feroz Amir Zafar a , Ishfaque A. Geelani a , Pravin Govardhane a , H. Girish a , D.V.S.L.N. Sharma a , V. Rajagopal a, *, Meenakshi Swain b , G. Swarnalatha b a Department of Urology, Apollo Hospitals, Hyderabad, India b Department of Histopathology, Apollo Hospitals, Hyderabad, India a r t i c l e i n f o Article history: Received 31 January 2013 Accepted 2 February 2013 Available online 9 February 2013 Keywords: Melanotic schwannoma Melanoma Nerve sheath tumor Adrenal schwannoma a b s t r a c t Introduction and objective: Melanotic schwannoma of adrenal gland is very rare entity with only two series and less than 100 cases reported in literature. We report a case of melanotic schwannoma. Case report: A 62 years old man, a resident of Tanzania presented with 6 months history of lower urinary tract symptoms (LUTS) for which he was evaluated and a right adrenal mass detected incidentally on ultrasound. He is known case of hypertension and hypothyroidism for which he is taking regular medications. On general examination pulse was 80/min, regular and BP was130/80 mm of Hg. Abdominal examination was unremarkable. MDCT examination revealed a right ad- renal mass (4 Â 4.8 cm) showing mild enhancement post contrast study with central area of relatively less enhancement showing no significant contrast washout on delayed study. The provisional diagnosis was ? pheochromocytoma, ? malignant. Functional study was found to be normal. Laparoscopic exploration was done and mass excised. Gross patho- logical examination showed a single globular circumscribed soft tissue mass measuring 4.5 Â 4 Â 3 cm and weighing 50 g with a nodular surface. The cut surface was black with a central golden brown area measuring around 2.2 Â 1.8 cm. A diagnosis of malignant melanoma was considered, however in view of circum- scription bland nuclei, absent of nucleoli, very few mitoses and presence of nerve fascicles; a diagnosis of melanotic schwannoma was favored. The patient was advised regular fol- low-up. Conclusion: Melanotic schwannoma of adrenal is a very rare entity and every effort should be made to rule out melanoma. Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved. 1. Introduction Melanotic schwannoma of the adrenals is a rare, accounting for only 1e3% of all schwannomas and only 1% of all retroperitoneal tumors.1,2 They are circumscribed but unen- capsulated, grossly pigmented tumor, composed of cells having the ultra structure and immunophenotype of Schwann cells that contain melanosomes and are reactive for * Corresponding author. E-mail address: raja_urol@rediffmail.com (V. Rajagopal). Available online at www.sciencedirect.com journal homepage: www.elsevier.com/locate/apme a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 7 7 e8 1 0976-0016/$ e see front matter Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved. http://dx.doi.org/10.1016/j.apme.2013.02.001
    • melanoma markers. Cytological atypia is not uncommon. The vast majority of non-psammomatous tumors affect spinal nerves and paraspinal ganglia, whereas the psammomatous lesions also involve autonomic nerves of viscera, such as the intestinal tract, adrenal and heart. About 50% of patients with psammomatous melanotic schwannoma have the rare inherited autosomal-dominant disorder Carney complex type Fig. 1 e CECT of abdomen & pelvis (coronal, saggital & transverse planes) showing the right adrenal mass (white arrow). Fig. 2 e CECT of abdomen & pelvis (coronal, saggital & transverse planes) showing the right adrenal mass (white arrow). Fig. 3 e CECT of abdomen & pelvis (coronal, saggital & transverse planes) showing the right adrenal mass (white arrow). Fig. 4 e Laparoscopic views of the darkly pigmented tumor (yellow arrow). a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 7 7 e8 178
    • I (CNC) characterized by facial pigmentation, Cardiac myx- oma, and endocrine overactivity. A correct preoperative diagnosis is difficult to make and these tumors are often misdiagnosed as other soft tissue lesions occurring in the retroperitoneum. Over 80 cases of one or more tumors have been reported in literature so far.3 We describe a case of melanotic schwan- noma of the adrenal gland. 2. Case presentation Sixty two years gentleman, resident of Tanzania presented with h/o lower urinary tract symptoms (LUTS) since >6 months and an incidentally detected right adrenal mass. He gave a past history of hypertension and hypothyroidism which was under control with medications. On clinical ex- amination, pulse e 80/min, BP e 130/80 mm of Hg. General examination e No pallor/icterus/cyanosis/clubbing/edema feet. P/A e soft, non-tender, no mass was palpable. In- vestigations revealed Hb-12 g%, TLC-4100/mm,3 S. creatinine e 1.2 mg%, RBS e 74 mg%, Metanephrines (24 h urine) e 1.0 mg/day, VMA (24 h urine) e 8.7/day, S. cortisol (dex- amethasone suppression test) e 0.56 mg/dl. CT scan (Figs. 1e3) showed a right adrenal mass (4 Â 4.8 cm) showing mild enhancement post contrast study with central area of rela- tively less enhancement showing no significant contrast wash out on delayed study (? pheochromocytoma/? malignant). At Laparoscopic exploration, a heterogeneous, darkly pig- mented (almost black) unencapsulated neoplasm was found in the right retroperitoneal space, medially displacing the inferior vena cava and clearly separated from the kidney and the renal vein, which are pushed downward. The tumor was completely excised (Figs. 4 and 5). The specimen was sent for histopathological examination. Fig. 5 e Laparoscopic views of the darkly pigmented tumor (yellow arrow). Fig. 6 e Resected specimen (pigmented). Fig. 7 e Cut section of the tumor (heterogeneous). Fig. 8 e Low power 103 H&E stain. a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 7 7 e8 1 79
    • 2.1. Gross description Single globular circumscribed soft tissue measuring 4.5 Â 4 Â 3 cm & weighing 50 g. External surface was nodular (Fig. 6). Cut surface was black with a central golden brown area measuring around 2.2 Â 1.8 cm (Fig. 7). 2.2. Microscopically Lobules and nests of tumor cells with large areas of necrosis and hemorrhage. The tumor cells are spindle shaped and ar- ranged in intersecting fascicles. Tumor cells have moderate ill defined fibrillary cytoplasm and have elongated bland vesic- ular nuclei with few showing nuclear grooves (Fig. 8). Abundant brownish black pigment is seen with in tumor cells and also within the histiocytes (Fig. 10). Few mitoses were seen averaging 1e2/10 hpf. Prominent nuclei typical of melanoma are not seen. Perl’s stain is positive in the pigment in hemosiderin laden macrophages and negative in pigment in tumor cells (Fig. 11). A few nerve fascicles are seen at the periphery adherent to the tumor (Fig. 9). 3. Discussion Melanotic schwannomas (MS) were first described in 1932 by Millar.4 These tumors are rare, with approximately 80 cases reported in the literature to date.3 Melanotic schwannomas are of neural crest origin proba- bly caused by the neoplastic proliferation of a common pre- cursor cell for both Schwann cells and melanocytes.5,6 Schwannoma is a neurogenic tumor usually arising between the third and sixth decades of life, with an equal predilection for men and women. It may occur in any organ or nerve trunk with the exception of cranial nerves I and II, which lack Schwann cells A preoperative diagnosis is very difficult to make because of the lack of typical imaging features (US, CT and MRI) that distinguish schwannomas from retroperitoneal malignancies.7 Even with accurate preoperative imaging and intraoperative frozen section analysis, the presence of a pos- sible malignancy cannot be excluded, so a definitive diagnosis can be obtained only through careful histological and immu- nohistochemical examination. Surgical excision is considered the treatment of choice for these tumors, which respond poorly to radiation and chemotherapy.8 We were unable to make a correct preoperative diagnosis. The following differ- ential diagnosis was considered: malignant melanoma, mel- anotic schwannoma, pigmented pheochromocytoma and leiomyosarcoma. There was a diagnostic dilemma and ma- lignant melanoma was strongly considered, however in a view of the circumscription, bland nuclei, absence of nucleoli, very few mitoses and the presence of nerve fascicles, a diagnosis of a melanotic schwannoma was finally favored. 4. Conclusion Melanotic schwannoma is a rare variant of schwannoma composed of melanin-producing cells with ultrastructural features of Schwann cells. Distinguishing between this tumor and malignant melanoma is important in planning manage- ment. Total resection should be performed. Appropriate long- term follow-up is needed for all melanotic schwannomas. Fig. 9 e 203 showing tumor attached to nerve fascicles. Fig. 10 e H&E (403) e Spindle cells running in fascicles with intracytoplasmic melanin pigment. Fig. 11 e Immunohistochemistry showing positive S 100. a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 7 7 e8 180
    • Conflicts of interest All authors have none to declare. r e f e r e n c e s 1. Ueda M, Okamoto Y, Ueki M. A pelvic retroperitoneal schwannoma arising in the right paracolpium. Gynecol Oncol. 1996;60:480e483. 2. Hayasaka K, Tanaka Y, Soeda S, Huppert P, Claussen CD. MR findings in primary retroperitoneal schwannoma. Acta Radiol. 1999;40:78e82. 3. Zhang HY, Yang GH, Chen HJ, et al. Clinicopathological, immunohistochemical, and ultrastructural study of 13 cases of melanotic schwannoma. Chin Med J (Engl). 2005;118(17):1451e1461. 4. Millar W. Malignant melanotic tumor of the ganglion cells arising from the thoracic sympathetic ganglion. J Pathol. 1932;35:351e357. 5. Vallat-Decouvelaere A-V, Wassef M, Lot G, et al. Spinal melanotic schwannoma: a tumour with poor prognosis. Histopathology. 1999;35:558e566. 6. Watson JC, Stratakis CA, Bryant-Greenwood PK, et al. Neurosurgical implications of Carney complex. J Neurosurg. 2000;92:413e418. 7. Hughes MJ, Thomas JM, Fisher C, Moskovic EC. Imaging features of retroperitoneal and pelvic schwannomas. Clin Radiol. 2005;60:886e893. 8. Daneshmand S, Youssefzadeh D, Chamie K, et al. Benign retroperitoneal schwannoma: a case series and review of the literature. Urology. 2003;62:993e997. a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 7 7 e8 1 81
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