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CORRESPONDENCE
Year : 2014  |  Volume : 10  |  Issue : 2  |  Page : 371-373

Aggressive angiomyxoma of greater omentum with pleural effusion in a young male


1 Department of Pulmonary Medicine, Burdwan Medical College and Hospital, Burdwan, India
2 Department of Pulmonary Medicine, NRS Medical College and Hospital, Shealdah, Kolkata, West Bengal, India
3 Department of General Surgery, NRS Medical College and Hospital, Shealdah, Kolkata, West Bengal, India

Date of Web Publication14-Jul-2014

Correspondence Address:
Kaushik Saha
Department of Pulmonary Medicine, First Lane, P. O. - Nimta, Kolkata - 700 049, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.136661

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 > Abstract 

Aggressive angiomyxoma is a rare, locally aggressive, myxoid mesenchymal neoplasm, preferentially arising in the pelvic and perineal regions of young adult females. It may also occur in males, in the inguinoscrotal area. Here we report a case of a young male, who presented with a firm, diffuse, nontender swelling in the abdomen and right-sided pleural effusion. Contrast-enhanced computed tomography (CT) of the abdomen showed the presence of a large, cystic mass, almost filling the entire abdominal cavity. On laparotomy, a huge mass, approximately 25 cm × 20 cm × 4 cm in size, arising from the greater omentum, with increased vascularity, and occupying the entire abdomen, was excised. Histopathological examination along with immunohistochemistry established the diagnosis of an aggressive angiomyxoma. Here, we report the case of an aggressive angiomyxoma, originating from the greater omentum, in a young male, associated with right-sided pleural effusion.

 > Abstract in Chinese 

1例年轻男性大网膜侵袭性血管肌瘤伴胸腔积液
摘要
侵袭性血管黏液瘤是一种罕见的局部侵袭性,黏液样间质肿瘤,好发于青年成年女性盆腔及会阴区。它也可能发生在男性阴囊区。本文报告一个年轻男性病例,他长了一个固定的,冗长的,无触痛的肿胀在腹部,并伴有右胸腔积液。CT显示腹部有一个巨大的,囊性肿块,几乎充满整个腹腔。剖腹探查发现一个约25厘米*20厘米*4厘米大小的肿块,,位于大网膜,血管分化良好,并占据整个腹腔,将其切除。组织病理学检查和免疫组化诊断为侵袭性血管黏液瘤。
关键词:侵袭性的,血管黏液瘤,大网膜,胸腔积液,年轻的男性


Keywords: Aggressive, angiomyxoma, greater omentum, pleural effusion, young male


How to cite this article:
Saha K, Sarkar S, Jash D, Chatterjee S, Saha AK. Aggressive angiomyxoma of greater omentum with pleural effusion in a young male. J Can Res Ther 2014;10:371-3

How to cite this URL:
Saha K, Sarkar S, Jash D, Chatterjee S, Saha AK. Aggressive angiomyxoma of greater omentum with pleural effusion in a young male. J Can Res Ther [serial online] 2014 [cited 2019 Nov 15];10:371-3. Available from: http://www.cancerjournal.net/text.asp?2014/10/2/371/136661


 > Introduction Top


Aggressive angiomyxoma is a type of soft tissue tumor, which constitutes a rare variety of benign neoplasm. It was first described in 1983, by Steeper. [1] It tends to occur predominantly in the pelvic and perineal regions of reproductive females. A few cases have been reported in males. The common site of location of the tumor is in the inguinal region, along the spermatic cord or scrotum, in males. Here, we report a rare case of aggressive angiomyxoma originating from the greater omentum, in a young male, presenting as an abdominal swelling.


 > Case report Top


A 17-year-old male was admitted with gradually increasing swelling of the abdomen for the last three months, with dry cough for the last one month. On clinical examination, there was a nontender, firm, diffuse swelling felt all over the abdomen. The respiratory system examination was suggestive of a right-sided pleural effusion. A large, predominantly cystic, space-occupying lesion was found on ultrasonography (USG) of the whole abdomen. Contrast-enhanced computed tomography (CT) of the abdomen showed the presence of a large, cystic mass, almost filling the entire abdominal cavity, including the pelvis [Figure 1]. The bowels were compressed and displaced by the mass. Mild ascites were also noted. Moderate right-sided pleural effusion was noted, along with underlying segmental atelectasis. Pleural fluid cytology was lymphocytic, exudative in nature, with an adenosine deaminase (ADA) level of 27.8. No malignant cell was detected on pleural fluid examination. CT-guided fine needle aspiration cytology (FNAC) of the abdominal mass showed a few mesothelial cells, blood elements, and amorphous material, with absence of malignant cells. The patient underwent laparotomy with complete excision of the mass. There was a presence of a huge mass, approximately 25 cm × 20 cm × 4 cm in size, with increased vascularity, occupying the entire abdomen, arising from the greater omentum, with omental fat loss in the residual areas, and mild adhesions to the proximal ileum and transverse colon. The wall of the mass was very friable, with clear fluid content inside the mass. The cut surface of the tumor had a grayish mucoid appearance. The histopathological examination showed that the tumor was composed of widely scattered spindle- and stellate-shaped cells, with an ill-defined cytoplasm, and variably sized, thin- and thick-walled vascular channels, lying in a myxoid stroma, rich in collagen fibers. The fibrous tissue was in the form of interlacing or parallel bands of collagen, with edema. No mitotic figure or lipoblast was found. The stroma was infiltrated by lymphocytes, plasma cells, and eosinophils [Figure 2]. Overall, the histopathological feature was suggestive of aggressive angiomyxoma. Immunohistochemistry was positive for vimentin and desmin, while it was negative for actin and myosin. Hence, a diagnosis of aggressive angiomyxoma was made.
Figure 1: Contrast-enhanced CT of the abdomen showing the presence of a large, cystic mass, almost filling the entire abdominal cavity including the pelvis

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Figure 2: Histopathology of the excised mass showing widely scattered spindle- and stellate-shaped cells, with an ill-defined cytoplasm, and variably sized thin- and thick-walled vascular channels lying in a myxoid stroma rich in collagen fibers, suggestive of aggressive angiomyxoma (H and E, ×50)

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 > Discussion Top


Aggressive angiomyxoma is a rare, locally aggressive myxoid mesenchymal neoplasm, preferentially arising in the pelvic and perineal regions of young adult females, most commonly in the reproductive age group. To date, to our knowledge, only 250 cases have been reported in literature. [2] Among them, 90% of the cases have been found in females. [3] Few cases were also reported among males, arising in inguinoscrotal area. The initial presentation varies from a painless, poorly localized, gelatinous mass in the vulva, to a pelvic mass found in imaging studies. The presenting features are nonspecific, such as, a palpable mass, with heaviness or discomfort in the lower abdomen. Angiomyxomas usually exhibit a slow, insidious growth pattern, a capacity for local infiltration, and a marked tendency for repeated local recurrence. An ultrasound is helpful in this regard and usually demonstrates a hypoechoic soft tissue mass that may even appear cystic. Imaging studies may give a clue to the diagnosis, but histopathological examination is required for confirmation of the diagnosis. Appearances on a CT are variable and include a well-defined homogenous mass, hypodense, relative to muscle, a hypoattenuating solid mass, with a swirling internal pattern with contrast, or a predominantly cystic mass with a solid component. [4] On gross examination, the cut surface of the tumor reveals a glistening, soft, homogenous appearance. The angiomyxomas are usually homogenous in consistency, with no nodularity. [1] Histologically, they are characterized by a hypocellular mesenchymal lesion, consisting of a sparse population of bland spindle and stellate cells scattered in athe background of a loose myxoid stroma, composed of wavy collagen fibrils. The cells have abundant wispy pink cytoplasm, with bland nuclei. There is no cytological atypia, no atypical mitotic features or discernible mitotic activity, and no evidence of coagulative tumor cell necrosis either. Vascular proliferation is also prominent. [5] A vast majority of cases demonstrate positivity for desmin in the myxoid bundles or stromal cells, while actin and CD34 may be variably positive. [6] Positive staining for estrogen and progesterone receptors lead to the suspicion of a role of hormones in the pathogenesis of angiomyxoma. The tumor is slow growing in nature, which is also evident histologically, by its benign nature. It is locally aggressive and tends to recur (36-72%) after resection. [7] Wide local excision was the treatment of choice in this patient. [8] Preoperative angiographic embolization, preoperative external beam irradiation, and intraoperative electron beam radiotherapy, are useful to decrease the chances of local recurrence. [8] As these tumors are likely to be hormone-dependent, GnRH analogs may be used for preoperative shrinkage of this tumor. [9] Recurrences generally occur in the first five years after primary surgery, about 70% in the first three years, but late recurrences up to 14 years have been reported. Follow-up should always be clinically supplemented with radiological investigations.

We wanted to report the case, as it is a very rare disease. Moreover in our case, the aggressive angiomyxoma originated from the greater omentum, in a young male. To our knowledge, only two cases of aggressive angiomyxoma arising from the greater omentum have been reported in literature. Also to add, only two cases of lung metastasis and one case of pleural effusion associated with aggressive angiomyxoma are available in the literature.

 
 > References Top

1.Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm. Am J Surg Pathol 1983;7:463-75.  Back to cited text no. 1
[PUBMED]    
2.Haldar K, Martinek IE, Kehoe S. Aggressive angiomyxoma: A case series and literature review. Eur J Surg Oncol 2010;36:335-9.  Back to cited text no. 2
    
3.Wiser A, Korach J, Gotlieb WH, Fridman E, Apter S, Ben-Baruch G. Importance of accurate preoperative diagnosis in the management of aggressive angiomyxoma: Report of three cases and review of the literature. Abdom Imaging 2006;31:383-6.  Back to cited text no. 3
    
4.Jeyadevan NN, Sohaib SA, Thomas JM, Jeyarajah A, Shepherd JH, Fisher C. Imaging features of aggressive angiomyxoma. Clin Radiol 2003;58:157-62.  Back to cited text no. 4
    
5.van Roggen JF, van Unnik JA, Briaire-de Bruijn IH, Hogendoorn PC. Aggressive angiomyxoma: A clinicopathological and immuno histochemical study of 11 cases with long-term follow-up. Virchows Arch 2005;446:157-63.  Back to cited text no. 5
    
6.Smith HO, Worrell RV, Smith AY, Dorin MH, Rosenberg RD, Bartow SA. Aggressive angiomyxoma of the female pelvis and perineum: Review of the literature. Gynecol Oncol 1991;42:79-85.  Back to cited text no. 6
    
7.Outwater EK, Marchetto BE, Wagner BJ, Siegelman ES. Aggressive angiomyxoma: Findings on CT and MR imaging. AJR Am J Roentgenol 1999;172:435-8.  Back to cited text no. 7
    
8.Nyam DC, Pemberton JH. Large aggressive angiomyxoma of the perineum and pelvis: An alternative approach. Report of a case. Dis Colon Rectum 1998;41:514-6.  Back to cited text no. 8
    
9.McCluggage WG, Jamieson T, Dobbs SP, Grey A. Aggressive angiomyxoma of the vulva: Dramatic response to gonadotropin-releasing hormone agonist therapy. Gynecol Oncol 2006;100:623-5.  Back to cited text no. 9
    


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