|Year : 2014 | Volume
| Issue : 2 | Page : 371-373
Aggressive angiomyxoma of greater omentum with pleural effusion in a young male
Kaushik Saha1, Supriya Sarkar2, Debraj Jash2, Sajib Chatterjee3, Asis Kumar Saha3
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 Publication||14-Jul-2014|
Department of Pulmonary Medicine, First Lane, P. O. - Nimta, Kolkata - 700 049, West Bengal
Source of Support: None, Conflict of Interest: None
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.
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
| > Introduction|| |
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.  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|| |
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|| |
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.  Among them, 90% of the cases have been found in females.  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.  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.  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.  A vast majority of cases demonstrate positivity for desmin in the myxoid bundles or stromal cells, while actin and CD34 may be variably positive.  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.  Wide local excision was the treatment of choice in this patient.  Preoperative angiographic embolization, preoperative external beam irradiation, and intraoperative electron beam radiotherapy, are useful to decrease the chances of local recurrence.  As these tumors are likely to be hormone-dependent, GnRH analogs may be used for preoperative shrinkage of this tumor.  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|| |
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[Figure 1], [Figure 2]