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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 6  |  Issue : 1  |  Page : 114-115

A rare case of adrenal rhabdomyosarcoma


Department of Surgical Oncology, Chittaranjan National Cancer Institute, Kolkata, West Bengal, India

Date of Web Publication29-Jun-2017

Correspondence Address:
Debraj Saha
61, Sovabazar Street, Kolkata - 700 005, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0513.209146

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  Abstract 

Rhabdomyosarcoma (RMS) of the adrenal gland is a very rare entity in medical literature. Very few case reports are available in the journals. In our case report, we have shared our clinical experience of coming across such a scenario where a 43-year-old woman presented with left-sided adrenal mass, later on proved to be pleomorphic RMS. To the best of our knowledge, this is the second case of pleomorphic RMS of the adrenal gland in an adult diagnosed by light microscopy and immunohistochemical stains.

Keywords: A rare experience, adrenal gland, rhabdomyosarcoma


How to cite this article:
Saha D, Majumder J, Chejara S, Basu A, Pramanik S, Das S. A rare case of adrenal rhabdomyosarcoma. Clin Cancer Investig J 2017;6:114-5

How to cite this URL:
Saha D, Majumder J, Chejara S, Basu A, Pramanik S, Das S. A rare case of adrenal rhabdomyosarcoma. Clin Cancer Investig J [serial online] 2017 [cited 2019 Nov 18];6:114-5. Available from: http://www.ccij-online.org/text.asp?2017/6/1/114/209146


  Introduction Top


Rhabdomyosarcoma (RMS) is a malignant soft tissue sarcoma that is believed to develop from primitive totipotent embryonic mesenchyme. RMS is a highly aggressive tumor with a tendency for advanced and disseminated disease early in its course. The condition is the most common soft tissue sarcoma in children. However, RMS in adults is an uncommon tumor that arises mainly in the large skeletal muscles.[1],[2],[3],[4] Pleomorphic RMS was first described by Stout in 1946.[5] More recent studies have reported that pleomorphic RMS is rare and occurs predominantly in adults. It can also be found attached to muscle tissue, wrapped around intestines, or in any anatomic location. It mostly occurs in areas naturally lacking in skeletal muscle, such as the head, neck, and genitourinary tract.


  Case Report Top


A 43-year-old lady presented in our outpatient department with mild left lower abdominal pain and discomfort for the last 6 months. Clinical examination revealed a painless retroperitoneal mass measuring 10 cm × 12 cm, occupying left hypochondrial, lumbar, umbilical quadrants of abdomen. The patient was neither hypertensive nor diabetic. Other physical examination was unremarkable. Patient had no history suggestive of diaphoresis, sweating, paroxysomal hypertension, and headache. Patient had no history of abdominal complains in the form of hematemesis, malena, vomiting. Computed tomography of whole abdomen showed a large well-defined heterogenous inhomogenously enhancing retroperitoneal space occupying lesion (SOL) measuring 14.8 cm × 10.5 cm [Figure 1]. The lesion has displaced bowel loops, spleen and pancreatic tail anteriorly, and left kidney inferiorly. Left adrenal gland is not seen separately. Fat planes adjacent to the lesion are intact. Right adrenal is normal.
Figure 1: Computed Tomography (CT abdomen) - Shows A large well defined heterogenous inhomogenously enhancing retroperitoneal SOL measuring (14.8x10.5) cm

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Ultrasonography-guided fine-needle aspiration biopsy from the retroperitoneal SOL was suggestive of adrenal cortical carcinoma. Value of biochemical 24 h urinary vanillylmandelic acid was normal. After exploratory laparotomy, we offered her left-sided adrenalectomy. Intraoperative period was uneventful. Postoperative histopathological analysis revealed that it was either adrenal cortical carcinoma or pheochromocytoma. On immunohistochemistry, it was favorable with the diagnosis of pleomorphic RMS as it was strongly positive to vimentin, desmin, myogenin, CD56 [Figure 2]. No expression of smooth muscle actin (SMA), synaptophysin (SYN), or S-100 protein was identified in the tumor tissue.
Figure 2: Immunohistochemistry-positive for vimentin

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  Discussion Top


In the current World Health Organization Classification of Soft Tissue and Bone Neoplasms, RMS is divided into three distinct subtypes, embryonic, alveolar, and pleomorphic.[6] RMS is a rare disease of the adrenal gland neoplasm, which predominantly occurs in adults. Charytonowicz et al.[7] suggest that RMS may arise from nonmuscle cells, including mesenchymal stem cells. Theoretically, RMS may affect any body part including the adrenal glands as shown in the present case. To date, only two RMS cases in the adrenal region have been described in English literature. Yi et al.[8] reported a case of alveolar RMS in the right adrenal region of a pediatric patient with a characteristic history of hypertension and fever. Katayama et al.[9] reported a case of RMS in the adrenal region of an elderly hypertensive patient. However, pleomorphic RMS of the adrenal gland in an adult has not been previously reported. In the present case, light microscopic examination revealed a malignant pleomorphic mesenchymal neoplasm, characterized mainly by the proliferation of atypical spindle cells and few epithelioid cells. Immunohistochemistry revealed positive staining for MyoD1, desmin, vimentin, and CD56. By contrast, no expression of SMA, SYN, or S-100 protein was identified in tumor tissue. A diagnosis of pleomorphic RMS was confirmed according to the clinical and pathological findings.


  Conclusion Top


The present study described a rare case of pleomorphic RMS in the left adrenal region based on the immunohistochemistry results. Due to the small number of described cases of adrenal gland RMS, inadequate information is available for evaluating the treatment procedure and the final prognosis of the patient. An accumulation of such cases and an improved understanding of the molecular biology driving RMS tumor behavior are required for further evaluation and research to identify the histogenesis of the condition. Primary pleomorphic RMS of the adrenal gland in an adult is a rare condition. To the best of our knowledge, this is the second case of pleomorphic RMS of the adrenal gland in an adult diagnosed by immunohistochemical staining.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Furlong MA, Mentzel T, Fanburg-Smith JC. Pleomorphic rhabdomyosarcoma in adults: A clinicopathologic study of 38 cases with emphasis on morphologic variants and recent skeletal muscle-specific markers. Mod Pathol 2001;14:595-603.  Back to cited text no. 1
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2.
Ogilvie CM, Crawford EA, Slotcavage RL, King JJ, Lackman RD, Hartner L, et al. Treatment of adult rhabdomyosarcoma. Am J Clin Oncol 2010;33:128-31.  Back to cited text no. 2
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3.
Stock N, Chibon F, Binh MB, Terrier P, Michels JJ, Valo I, et al. Adult-type rhabdomyosarcoma: Analysis of 57 cases with clinicopathologic description, identification of 3 morphologic patterns and prognosis. Am J Surg Pathol 2009;33:1850-9.  Back to cited text no. 3
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4.
Sultan I, Qaddoumi I, Yaser S, Rodriguez-Galindo C, Ferrari A. Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: An analysis of 2,600 patients. J Clin Oncol 2009;27:3391-7.  Back to cited text no. 4
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5.
Stout AP. Rhabdomyosarcoma of the skeletal muscles. Ann Surg 1946;123:447-72.  Back to cited text no. 5
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6.
Fletcher CD, Unni KK, Mertens F, editors. Pathology and Genetics of Tumors of Soft Tissue and Bone Soft Tissue Tumors. Lyon: IARC Press; 2002. p. 146-53.  Back to cited text no. 6
    
7.
Charytonowicz E, Cordon-Cardo C, Matushansky I, Ziman M. Alveolar rhabdomyosarcoma: Is the cell of origin a mesenchymal stem cell? Cancer Lett 2009;279:126-36.  Back to cited text no. 7
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8.
Yi X, Long X, Xiao D, Zai H, Li Y. Rhabdomyosarcoma in adrenal region of a child with hypertension and fever: A case report and literature review. J Pediatr Surg 2013;48:e5-8.  Back to cited text no. 8
    
9.
Katayama A, Otsuka F, Takeda M, Miyoshi T, Nakamura E, Inagaki K, et al. Rhabdomyosarcoma discovered in the adrenal region of an elderly hypertensive patient. Hypertens Res 2011;34:784-6.  Back to cited text no. 9
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    Figures

  [Figure 1], [Figure 2]



 

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