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==Abstract==
 
==Abstract==
  
We present the clinical case of a 74-year-old woman with an urachal tumor diagnosed in 2004. The patient underwent a partial cystectomy of the dome with remotion of the urachal remnant and the umbilicus. She had been clinical well until 2011 when she had a kidney metastasis from the urachal tumor.
+
An angiomyofibroblastoma-like tumor is a rare mesenchymal tumor that arises in the scrotum, inguinal region or perineum in men. It is usually described as superficial, originating in subcutaneous tissues. In the present case we found an angiomyofibroblastoma-like tumor with a different clinical presentation. We describe a benign tumor that mimicked a testicular tumor clinically and in imaging exams. This tumor was herniated to the scrotum through an indirect inguinal hernia that had arisen from the omentum and the mesenteric/splanchnic circulation; these were the only structures it was linked to. Surgical excision is the recommended treatment and cure is obtained in most cases.
 
+
Urachal tumors are very rare, comprising 0.17-0.34% of all bladder cancers. The most common sites of distant metastases are: lung, lymph nodes, bone, intestine, brain, liver, peritoneum, skin and spine. We describe an extremely rare case of a renal metastasis from an urachal cancer, seven years after the initial diagnosis.
+
 
+
Usually, patients with localized disease have a good prognosis when treated with surgery. The prognosis gets worse for patients with metastases or local recurrence because a standard chemotherapy regimen does not exist.
+
  
 
==Resumo==
 
==Resumo==
  
Apresentamos o caso clinico de uma doente com 74 anos diagnosticada com tumor do úraco em 2004. A doente foi submetida a cistectomia parcial da cúpula vesical com excisão do úraco e do umbigo. Esteve clinicamente bem até 2011 quando lhe foi diagnosticada uma metástase renal do tumor do úraco.
+
Um angiomiofibroblastoma é um tumor mesenquimatoso raro que, nos homens, se desenvolve ao nível do escroto, da região inguinal ou do períneo. Habitualmente este tumor é superfi e origina-se no tecido celular subcutâneo. No presente caso encontrámos um angiangiomiofibroblastoma com uma apresentação clinica diferente. Descrevemos um tumor benigno que, do ponto de vista clinico e imagiológico, mimetizava um tumor do testículo. Este tumor encontrava-se ligado aos vasos da circulação esplâncnica/mesentérica e ao epiploon e fazia herniação para o escroto através de uma volumosa hérnia inguinal. A ressecção cirúrgica é o tratamento recomendado para estes tumores, com a cura a ser obtida na maioria dos casos.
 
+
Os tumores do úraco são muito raros, compreendendo 0,17-0,34% de todos os tumores da bexiga. Os locais mais comuns de metastização à distância são: pulmão, gânglios linfáticos, osso, intestino, fígado, peritoneu, pele e coluna. Descrevemos um caso extremamente raro de uma metástase renal de um adenocarcinoma do úraco sete anos após o diagnóstico inicial.
+
 
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Habitualmente, os doentes com doença localizada apresentam um bom prognóstico quando tratados com cirurgia. O diagnóstico é pior para doentes com metástases ou recorrência local uma vez que ainda não existe um regime de quimioterapia estabelecido.
+
  
 
==Keywords==
 
==Keywords==
  
Hematuria ; Kidney ; Metastasis ; Neoplasm ; Urachus
+
Angiomyofibroblastomalike tumor ; Inguinal hernia ; Mesenchymal tumor ; Testicular neoplasms
  
 
==Palavras-chave==
 
==Palavras-chave==
  
Hematúria ; Rim ; Mestástase ; Neoplasia ; Úraco
+
Tumor angiomiofibroblastoma like ; hérnia inguinal ; tumor mesenquimatoso ; tumor do testículo
  
 
==Introduction==
 
==Introduction==
  
Urachal adenocarcinomas are very rare tumors, usually diagnosed at an advanced stage because the lack of symptoms. We present a rare case of a renal metastasis developed seven years after the first treatment.
+
Angiomyofibroblastoma-like tumor is a rare mesenchymal tumor that occurs in men. Its name is based on similarities with the female angiomyofibroblastoma. The histogenesis of this tumor is the subject of speculation, with some evidence suggesting an origin from a perivascular mesenchymal cell. We describe an angiomyofibroblastoma-like tumor with a different clinical presentation and probably a different origin compared to cases described previously.
  
 
==Case report==
 
==Case report==
  
We present the case of a 74-year-old woman that was sent to the urologist in 2003 to investigate gross hematuria. She did a computed tomography (CT) scan that showed a proliferative mass of the anterior bladder wall. The cystoscopic evaluation confi a lesion at the bladder dome. The lesion was ressected and the histology revealed infiltration of the bladder mucosa by mucinous adenocarcinoma.
+
A 37-year-old man with no relevant medical history presented with an increased volume of the right testicle with a two-year evolution.
  
Gynecology and General Surgery excluded secondary adenocarcinoma of the bladder.
+
Clinically, in the right scrotum we palpated a tender, homogeneous and nonpainful mass of approximately 10 cm; the epididymis and testicle could not be isolated from this mass. No pathological inguinal nodes were detected.
  
A partial cystectomy of the dome was performed with remotion of the urachal ''remnant''  and the umbilicus. The histology revealed infiltration of the bladder mucosa by mucinous urachal adenocarcinoma ( [[#fig0005|Fig. 1]] ).
+
Ultrasonography detected a pronounced increase in the volume of the right testicle, with a 5.6 cm cephalocaudal diameter, hipoecoic and diffusely heterogeneous; these aspects suggested an expansive lesion.
 +
 
 +
A Computed Tomography Scan of the pelvis and abdomen revealed a right testicle partially visualized with increased dimensions embedded in the context of a proliferative mass; the fat around the spermatic cord was prominent. Peri-testicular vascular ingurgitation was also present that seemed to have a connection with the splanchnic/mesenteric circulation.
 +
 
 +
Tumor markers for testicular cancer, β-HCG and α-FP, were within the normal range.
 +
 
 +
We assumed the patient had a testicular tumor and he was proposed to radical orchiectomy.
 +
 
 +
During the surgery, contrary to expectations, it was ascertained that inside the scrotum the patient had a healthy right testicle and spermatic cord together with an indirect inguinal hernia whose sac surrounded the large mass ([[#fig0005|Fig. 1]] ).
  
 
<span id='fig0005'></span>
 
<span id='fig0005'></span>
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[[Image:draft_Content_626463816-1-s2.0-S2341402215000087-gr1.jpg|center|376px|Infiltration of the bladder mucosa by mucinous adenocarcinoma. ...]]
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[[Image:draft_Content_626463816-1-s2.0-S2341402215000099-gr1.jpg|center|376px|Appearance of the structures inside the right scrotum of the patient during the ...]]
  
  
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Figure 1.
 
Figure 1.
  
Infiltration of the bladder mucosa by mucinous adenocarcinoma. (Hematoxylin-eosin, 40×).
+
Appearance of the structures inside the right scrotum of the patient during the surgery; inside the right scrotum the patient had a healthy right testicle (thin white arrow) and spermatic cord together with an indirect inguinal hernia which sac (thin black arrow) was surrounding the big mass (thick black arrow); this mass was linked to the epiploon and splanchnic/mesenteric circulation (thick white arrow).
  
 
</span>
 
</span>
 
|}
 
|}
  
The follow-up was maintained and the patient had been clinically well until November 2011, when the CT scan of the abdomen and pelvis revealed a renal mass involving the lower pole of the left kidney. This mass had 75×41×54 mm and its appearance was suspicious for a proliferative lesion. There was no evidence of lymphadenopathies.
+
The mass inside the herniary sac was completely separated from it and only fixed to a small portion of the omentum and vessels; this portion was ligated, the mass was completely removed and the indirect inguinal hernia was corrected.
  
The patient underwent a laparoscopic radical nephrectomy of the left kidney on January 2012.
+
This tumor measured 12 × 9.5 × 7.5 cm and had a firm to soft consistency. Other macroscopic features included a solid cut surface, grey, with a brown/hemorrhagic stipple, and a central cystic hemorrhagic area of about 5 cm.
  
The histology showed infiltration of the renal parenchyma from the Gerota fascia to the calyces by mucinous adenocarcinoma ([[#fig0010|Fig. 2]] ). Imunohistochemical staining showed that cells were positive to CK20 but negative to CK7. Neural invasion was present and the margins were positive. The histologic exam concluded secondary infiltration of the kidney by mucinous adenocarcinoma similar to the analyses performed in 2003.
+
Microscopically, the tumor was a mesenchymal, spindled hypocellular proliferation, with cytonuclear atypia sparse or absent. These cells were in a stromal matrix containing small to medium sized blood vessels, composed of thick, eosinophilic collagen fibers with an admixture of thinner and lighter collagen fibers. Necrosis or mitotic figures were absent ([[#fig0010|Fig. 2]] ). Immunohistochemical analysis showed that the neoplastic cells reacted positively with vimentin ([[#fig0015|Fig. 3]] ) and focally with desmin. Immunoreactivity was negative for: smooth muscle actin, CD117, B-catenin, ALK, CKAE1-AE3, CA5.2, Calretin, WT1, trombomodulin, S100, CD68, CD99, bcl2 and IgG4.
  
 
<span id='fig0010'></span>
 
<span id='fig0010'></span>
Line 68: Line 68:
  
  
[[Image:draft_Content_626463816-1-s2.0-S2341402215000087-gr2.jpg|center|376px|Infiltration of the renal parenchyma by mucinous adenocarcinoma. ...]]
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[[Image:draft_Content_626463816-1-s2.0-S2341402215000099-gr2.jpg|center|376px|Photomicrograph of the tumor reveals a mesenchymal, spindled hypocellular ...]]
  
  
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Figure 2.
 
Figure 2.
  
Infiltration of the renal parenchyma by mucinous adenocarcinoma. (Hematoxylin-eosin, 40×).
+
Photomicrograph of the tumor reveals a mesenchymal, spindled hypocellular proliferation in a stromal matrix, containing small to medium size blood vessels, composed by thick, eosinophilic collagen fibers (white arrow) with an admixture of more thin and light collagen fibers (black arrow) (hematoxylin-eosin, ×100).
  
 
</span>
 
</span>
 
|}
 
|}
  
The gynecologic exam and the colonoscopy did not reveal alterations. It was concluded that the kidney mass was a metastasis from the urachal tumor.
+
<span id='fig0015'></span>
 
+
On May 2012, the patient had an episode of intestinal subocclusion and was admitted in the General Surgerys Department. During this period she underwent a virtual colonoscopy that did not reveal polipoid images. The CT scan of the thorax, abdomen and pelvis showed a latero-aortic lymph node and a heterogeneous lesion in the renal fossa sugestive of local relapse.
+
 
+
The serum levels of carcinoembryonic antigen (CEA) were elevated.
+
 
+
With evidence of disease progression the patient was sent to the Oncologist. She began salvage chemotherapy with ''Gemcitabine'' , once a week.     
+
 
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A CT scan performed on February 2013 showed volume reduction of the residual mass and of the latero-aortic lymph node. However it was also documented progression of the disease with identification of a lumbar vertebral sinking.
+
 
+
The patient died on March 2013.
+
 
+
==Discussion==
+
 
+
Adenocarcinoma of the bladder comprises less than 2% of all bladder carcinomas. They could arise from the bladder proper or from the urachus, or could be an extension of adenocarcinoma from adjacent organs or metastases from a distant organ.[[#bib0005|<sup>1</sup>]]
+
 
+
The histological differentiation between vesical, urachal and metastatic adenocarcinoma is difficult and requires correlation of clinical and pathological findings.<sup>[[#bib0005|1]]  ;  [[#bib0010|2]]</sup>
+
 
+
The immunostaining with CK7 and CK20 is used in surgical pathology to help determine the origin of epithelial neoplasms. However, by itself it does not provide sufficient specifi ty to allow accurate distinction between secondary colorectal adenocarcinomas and primary bladder adenocarcinomas.[[#bib0015|<sup>3</sup>]]
+
 
+
In order to diagnose an urachal cancer some criteria have to be fulfilled ([[#tbl0005|Table 1]] ). Some investigators consider any patient presenting with an enteric-type adenocarcinoma of the bladder, at the bladder dome or elsewhere in the midline as having an urachal tumor until proven otherwise.[[#bib0020|<sup>4</sup>]]
+
 
+
<span id='tbl0005'></span>
+
 
+
{| class="wikitable" style="min-width: 60%;margin-left: auto; margin-right: auto;"
+
|+
+
 
+
Table 1.
+
 
+
MD Anderson Cancer Centre Criteria for the diagnosis of urachal cancer.
+
  
 +
{| style="text-align: center; border: 1px solid #BBB; margin: 1em auto; max-width: 100%;"
 
|-
 
|-
 +
|
  
| Location in the bladder dome or elsewhere in the midline of the bladder
 
|-
 
  
| Sharp demarcation between tumor and normal surface epithelium
+
[[Image:draft_Content_626463816-1-s2.0-S2341402215000099-gr3.jpg|center|376px|Immunohistochemical analysis showing that the neoplastic cells reacted ...]]
|-
+
  
| Supportive criteria
 
|-
 
  
| Enteric-type histology
 
 
|-
 
|-
 +
| <span style="text-align: center; font-size: 75%;">
  
| Absence of urothelial dysplasia
+
Figure 3.
|-
+
  
| Absence of cystitis cystic or cystitis glandularis transitioning to the tumor
+
Immunohistochemical analysis showing that the neoplastic cells reacted positively with vimentin. (vimentin, ×40).
|-
+
  
| Absence of primary adenocarcinoma of another organ
+
</span>
 
|}
 
|}
  
Urachal tumors are very rare neoplasms that represent 0.01% of all cancers[[#bib0025|<sup>5</sup>]]  and 0.17-0.34% of all bladder cancers.[[#bib0030|<sup>6</sup>]]
+
The microscopic features and immunohistochemical profile were very suggestive that this mass could be an angiomyofibroblastoma-like tumor of the male genital tract.
  
The majority of cases are described in ''men'' , with many reports indicating a median age at diagnosis of approximately 50-60 years. <sup>[[#bib0025|5]] ; [[#bib0035|7]]  ;  [[#bib0040|8]]</sup>
+
''After two years of follow-up, there was no evidence of recurrence'' .    
  
A systematic review of the literature reveals a 5-year survival rate of less than 25%,<sup>[[#bib0025|5]]  ;  [[#bib0030|6]]</sup>  although some studies realized in Mayo Clinic revealed a 5-year survival rate of 43-49%.<sup>[[#bib0035|7]]  ;  [[#bib0040|8]]</sup>
+
==Discussion==
  
Patients with this kind of tumor most commonly present with hematuria.<sup>[[#bib0030|6]] ; [[#bib0035|7]]  ;  [[#bib0040|8]]</sup>  Another symptoms described by different authors are: mucinuria, palpable mass in the lower abdomen, bacteriuria, pain, disuria and umbilical discharge.<sup>[[#bib0020|4]] ; [[#bib0030|6]]  ;  [[#bib0035|7]]</sup>
+
Fletcher et al.[[#bib0005|<sup>1</sup>]] in 1992 described a new type of mesenchymal tumor named angiomyofibroblastoma that arose in the vulva and was different from aggressive angiomyxoma, which was locally invasive.[[#bib0010|<sup>2</sup>]]
  
Owing to lack of early symptoms, the cancer usually presents at an advanced stage.<sup>[[#bib0020|4]] ; [[#bib0025|5]] ; [[#bib0045|9]]  ;  [[#bib0050|10]]</sup>
+
According to the authors[[#bib0005|<sup>1</sup>]]  the designation angiomyofibroblastoma was based on the two integral components of this tumor: the blood vessels and the stroma; this tumor is composed of relatively bland spindled appearing cells and a loosely collagenous stroma with numerous small and medium sized vessels.<sup>[[#bib0015|3]] ; [[#bib0020|4]] ; [[#bib0025|5]] ; [[#bib0030|6]]  ;  [[#bib0035|7]]</sup> Vessels are usually rounded but occasionally compressed, with perivascular fibrosis; intralesional fat may be present.<sup>[[#bib0035|7]] ; [[#bib0040|8]]  ;  [[#bib0045|9]]</sup>  There are differences between the clinical forms of these tumors in men and women.     
  
The cystocopy usually shows a mass at the dome of the bladder or anterior wall which could be described in appearance as a polypoid or ulcerated lesion.<sup>[[#bib0030|6]]  ;  [[#bib0035|7]]</sup>
+
Concerning immunohistochemical analysis, angiomyofibroblastoma reacts positively and diffusely with vimentin<sup>[[#bib0005|1]] ; [[#bib0025|5]] ; [[#bib0030|6]] ; [[#bib0035|7]] ; [[#bib0045|9]]  ;  [[#bib0050|10]]</sup>  and has variable expression of desmin,<sup>[[#bib0005|1]] ; [[#bib0020|4]] ; [[#bib0025|5]] ; [[#bib0030|6]] ; [[#bib0035|7]] ; [[#bib0045|9]]  ;  [[#bib0050|10]]</sup>  CD34,<sup>[[#bib0005|1]] ; [[#bib0015|3]] ; [[#bib0020|4]] ; [[#bib0025|5]] ; [[#bib0030|6]] ; [[#bib0035|7]]  ;  [[#bib0040|8]]</sup>  smooth muscle actin<sup>[[#bib0025|5]] ; [[#bib0035|7]] ; [[#bib0040|8]]  ;  [[#bib0050|10]]</sup>  estrogen and progesterone receptors.<sup>[[#bib0025|5]] ; [[#bib0035|7]] ; [[#bib0040|8]]  ;  [[#bib0045|9]]</sup>
  
Like other enteric-type adenocarcinomas, urachal tumors may have detectable serum levels of CEA, CA125 and cancer antigen 19-9. These serum markers could be helpful in evaluating response to systemic chemotherapy.<sup>[[#bib0020|4]]  ;  [[#bib0050|10]]</sup>
+
The histogenesis of angiomyofibroblastoma is a subject of speculation,<sup>[[#bib0015|3]]  ;  [[#bib0045|9]]</sup>  but Laskin et al. consider that derivation of this tumor from a perivascular mesenchymal cell, with a capacity for fatty and myofibroblastic differentiation under environmental influences, cannot be excluded.<sup>[[#bib0035|7]]  ;  [[#bib0045|9]]</sup> This progenitor cell could be related to the CD-34 positive fibroblast-like cells that normally exist around the vessels. Theoretically, this cell may lose its ability to express CD-34 with myofibroblastic differentiation,[[#bib0035|<sup>7</sup>]]  in male angiomyofibroblastoma.     
  
The gold standard of treatment is the surgery that includes an en bloc resection of the urachal ligament and umbilicus with a complete or partial cystectomy.<sup>[[#bib0020|4]] ; [[#bib0030|6]]  ;  [[#bib0050|10]]</sup>  Sampling lymph nodes allows a better staging of the disease.[[#bib0035|<sup>7</sup>]]
+
Angiomyofibroblastoma-like tumor is a rare mesenchymal tumor that occurs in men in the scrotum,<sup>[[#bib0015|3]] ; [[#bib0020|4]] ; [[#bib0035|7]] ; [[#bib0040|8]]  ;  [[#bib0050|10]]</sup>  inguinal region,<sup>[[#bib0015|3]] ; [[#bib0020|4]] ; [[#bib0035|7]] ; [[#bib0040|8]]  ;  [[#bib0050|10]]</sup>  and perineum.[[#bib0015|<sup>3</sup>]]  This tumor was first described in 1997 as a cellular angiofibroma; its name was changed to angiomyofibroblastoma-like tumor by Laskin et al. in 1998[[#bib0035|<sup>7</sup>]] due to its similarities with the angiomyofibroblastoma tumor described in women in 1992.<sup>[[#bib0015|3]]  ;  [[#bib0040|8]]</sup>
  
Some authors describe as risk factors associated with recurrence after surgery: positive margins, lymph node or other metastases at the time of surgery, tumor grade and failure to resect the umbilicus.<sup>[[#bib0020|4]] ; [[#bib0025|5]]  ;  [[#bib0040|8]]</sup>
+
Angiomyofibroblastoma-like tumor is a superficial, well marginated and indolent tumor,<sup>[[#bib0015|3]] ; [[#bib0030|6]] ; [[#bib0035|7]] ; [[#bib0040|8]]  ;  [[#bib0050|10]]</sup>  ranging in size from to 14 cm.<sup>[[#bib0035|7]]  ;  [[#bib0050|10]]</sup>  It is usually asymptomatic but is occasionally associated with pain.<sup>[[#bib0015|3]] ; [[#bib0025|5]] ; [[#bib0035|7]] ; [[#bib0040|8]] ; [[#bib0045|9]]  ;  [[#bib0050|10]]</sup>  The majority of cases described occur during the fifth to eight decades.<sup>[[#bib0015|3]] ; [[#bib0020|4]]  ;  [[#bib0030|6]]</sup>
  
Local recurrence after surgery include: pelvic lymph nodes, peritoneum and omentum. The most common sites of distant metastases are: lung, lymph nodes, bone, intestine, brain, liver, peritoneal carcinomatosis, skin and spine.<sup>[[#bib0020|4]] ; [[#bib0035|7]]  ;  [[#bib0040|8]]</sup>  The rare distant metastases are: scalp, pleural effusion, adrenal, abdominal wall, pancreas and orbit.[[#bib0020|<sup>4</sup>]]
+
In our case study we have an indolent and superfi 12 cm tumor that appears in the scrotum, all characteristics of an angiofibroblastoma-like tumor; but, instead of arising from the subcutaneous tissues as usual, it seems to have developed from an atypical structure for this type of tumor, the omentum and mesenteric/splanchnic circulation, the only structures linked to the mass; it was identifi in the scrotum only because it was herniated through an indirect inguinal hernia.
  
Surgical resection of local tumor recurrences can be curative, but the role of radiation therapy and chemotherapy are unclear.[[#bib0040|<sup>8</sup>]]
+
Mesenchymal tumors are described as having a relationship with inguinal hernias,<sup>[[#bib0010|2]]  ;  [[#bib0030|6]]</sup> Some authors believe that the angiomyofibroblastoma-like tumor, could clinically simulate a hernia or a hydrocele,<sup>[[#bib0035|7]] ; [[#bib0045|9]]  ;  [[#bib0050|10]]</sup>  but in the available literature we ''do not''  find a description of this type of tumor herniated to the scrotum or linked to the omentum.     
  
There is no standard chemotherapy regimen and the choice of regimens has been based on case reports and single institution experiences.[[#bib0050|<sup>10</sup>]]
+
The recommended treatment for these tumors is surgical excision.<sup>[[#bib0015|3]] ; [[#bib0030|6]] ; [[#bib0040|8]]  ;  [[#bib0050|10]]</sup> The lesion is usually non recurrent,<sup>[[#bib0005|1]] ; [[#bib0040|8]] ; [[#bib0045|9]]  ;  [[#bib0050|10]]</sup>  with only one case of recurrence described thirteen years after surgery.[[#bib0035|<sup>7</sup>]]
  
The standard chemotherapy for this type of cancer should include a taxane or a regimen used to treat gastrointestinal malignances as opposed to standard transicional cell carcinoma regimens which have not shown much efficacy.[[#bib0050|<sup>10</sup>]]
+
In conclusion, we describe an angiomyofibroblastoma-like tumor with a different clinical presentation and probably a different origin compared to the cases described before; in this case report the tumor had a subcutaneous presentation simulating a testicular tumor but apparently did not arise from the subcutaneous tissues of the scrotum, perineum or inguinal region as usual<sup>[[#bib0015|3]] ; [[#bib0020|4]] ; [[#bib0035|7]] ; [[#bib0040|8]]  ;  [[#bib0050|10]]</sup> but from the omentum and the vessels of the mesenteric circulation, the only structures linked to the mass.    
 
+
The clinical trial with Gemcitabine+fluorouracil+leucovorin+cisplatin (Gem-FLP) is showing promise and may provide a chemotherapy standard.<sup>[[#bib0020|4]]  ;  [[#bib0050|10]]</sup>
+
 
+
''Usually, patients with localized disease have a good prognosis when treated with surgery. The prognosis gets worse for patients with metastases or local recurrence as we show with this case'' .     
+
 
+
We described a renal metastasis from urachal cancer. This clinical case shows that positive margins after surgery may represent a risk factor to recurrence. Given the rarity of urachal cancer, all new findings are important to improve the knowledge in this area. The creation of randomized follow-up studies with collaboration of different institutions is necessary to investigate the role of adjuvant and salvage therapies and establish a standard regimen.
+
  
 
==Ethical disclosures==
 
==Ethical disclosures==
  
'''Protection of human and animal subjects.'''  The authors declare that the procedures followed were in accordance with the regulations of the relevant clinical research ethics committee and with those of the Code of Ethics of the World Medical Association (Declaration of Helsinki).       
+
'''Protection of human and animal subjects.'''  The authors declare that no experiments were performed on humans or animals for this study.       
  
'''Confidentiality of data.'''  The authors declare that they have followed the protocols of their work center on the publication of patient data.       
+
'''Right to privacy and informed consent.'''  The authors declare that no patient data appear in this article. The authors declare that no patient data appear in this article.       
  
'''Right to privacy and informed consent.'''  The authors declare that no patient data appear in this article.       
+
'''Right to privacy and informed consent.'''  The authors declare that no patient data appear in this article.The authors declare that no patient data appear in this article.       
  
 
==Conflicts of interest==
 
==Conflicts of interest==
  
 
The authors have no conflicts of interest to declare.
 
The authors have no conflicts of interest to declare.
 
==Acknowledgments==
 
 
We acknowledge the Pathology Department of Hospital Curry Cabral for the figures.
 
  
 
==References==
 
==References==
  
 
<ol style='list-style-type: none;margin-left: 0px;'><li><span id='bib0005'></span>
 
<ol style='list-style-type: none;margin-left: 0px;'><li><span id='bib0005'></span>
[[#bib0005|1]] A. Pantuck, E. Bancila, K. Das, ''et al.''; Adenocarcinoma of the urachus and bladder expresses a unique colonic epithelial epitope: an immunohistochemical study; J Urol., 158 (1997), pp. 1722–1727</li>
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[[#bib0005|1]] C.D.M. Fletcher, W.Y.W. Tsang, C. Fisher, K.C. Lee, J.K.C. Chan; Angiomyofibroblastoma of the vulva. A benign neoplasm distinct from aggressive angiomyxoma; Am J Surg Pathol., 16 (1992), pp. 373–382</li>
 
<li><span id='bib0010'></span>
 
<li><span id='bib0010'></span>
[[#bib0010|2]] S. Roy, A. Parwani; Adenocarcinoma of the urinary bladder; Arch Pathol Lab Med., 135 (2011), pp. 1601–1605</li>
+
[[#bib0010|2]] R.J. Clatch, W.K. Drake, J.G. Gonzalez; Agressive angiomyxoma in man. A report of two cases associated with inguinal hernias; Arch Pathol Lab Med., 117 (1993), pp. 911–913</li>
 
<li><span id='bib0015'></span>
 
<li><span id='bib0015'></span>
[[#bib0015|3]] H. Wang, D. Lu, L. Yerian, ''et al.''; Immunohistochemical distinction between primary adenocarcinoma of the bladder and secondary colorectal adenocarcinoma; Am J Surg Pathol., 25 (2001), pp. 1380–1387</li>
+
[[#bib0015|3]] M. Maruyama, T. Yoshizako, H. Kitagaki, A. Araki, M. Igawa; Magnetic resonance imaging features of angiomyofibroblastomalike tumor of the scrotum with pathologic correlates; Clin Imaging., 36 (2012), pp. 632–635</li>
 
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<li><span id='bib0020'></span>
[[#bib0020|4]] A. Siefker-Radtke; Urachal carcinoma: surgical and chemotherapeutic options; Expert Rev Anticancer Ther., 6 (2006), pp. 1715–1721</li>
+
[[#bib0020|4]] K. Miyajima, S. Hasegawa, Y. Oda,  ''et al.''; Angiomyofibroblastomalike tumor (cellular angiofibroma) in the male inguinal region; Radiat Med., 25 (2007), pp. 173–177</li>
 
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<li><span id='bib0025'></span>
[[#bib0025|5]] A. Tsiouris, H.U. Ahmed, N. Kumar, A.M. Kaisary; Urachal tumour: clinical and radiological features of a poorly understood carcinoma; Ann R Coll Surg Engl., 89 (2007), pp. W17–W18</li>
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[[#bib0025|5]] A. Blel, A. Mekni, M. Bel Haj Salah, ''et al.''; Paratesticular angiomyofibroblastoma: case report and review of the literature; Pathologica., 100 (2008), pp. 489–491</li>
 
<li><span id='bib0030'></span>
 
<li><span id='bib0030'></span>
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Latest revision as of 11:30, 11 April 2017

Abstract

An angiomyofibroblastoma-like tumor is a rare mesenchymal tumor that arises in the scrotum, inguinal region or perineum in men. It is usually described as superficial, originating in subcutaneous tissues. In the present case we found an angiomyofibroblastoma-like tumor with a different clinical presentation. We describe a benign tumor that mimicked a testicular tumor clinically and in imaging exams. This tumor was herniated to the scrotum through an indirect inguinal hernia that had arisen from the omentum and the mesenteric/splanchnic circulation; these were the only structures it was linked to. Surgical excision is the recommended treatment and cure is obtained in most cases.

Resumo

Um angiomiofibroblastoma é um tumor mesenquimatoso raro que, nos homens, se desenvolve ao nível do escroto, da região inguinal ou do períneo. Habitualmente este tumor é superfi e origina-se no tecido celular subcutâneo. No presente caso encontrámos um angiangiomiofibroblastoma com uma apresentação clinica diferente. Descrevemos um tumor benigno que, do ponto de vista clinico e imagiológico, mimetizava um tumor do testículo. Este tumor encontrava-se ligado aos vasos da circulação esplâncnica/mesentérica e ao epiploon e fazia herniação para o escroto através de uma volumosa hérnia inguinal. A ressecção cirúrgica é o tratamento recomendado para estes tumores, com a cura a ser obtida na maioria dos casos.

Keywords

Angiomyofibroblastomalike tumor ; Inguinal hernia ; Mesenchymal tumor ; Testicular neoplasms

Palavras-chave

Tumor angiomiofibroblastoma like ; hérnia inguinal ; tumor mesenquimatoso ; tumor do testículo

Introduction

Angiomyofibroblastoma-like tumor is a rare mesenchymal tumor that occurs in men. Its name is based on similarities with the female angiomyofibroblastoma. The histogenesis of this tumor is the subject of speculation, with some evidence suggesting an origin from a perivascular mesenchymal cell. We describe an angiomyofibroblastoma-like tumor with a different clinical presentation and probably a different origin compared to cases described previously.

Case report

A 37-year-old man with no relevant medical history presented with an increased volume of the right testicle with a two-year evolution.

Clinically, in the right scrotum we palpated a tender, homogeneous and nonpainful mass of approximately 10 cm; the epididymis and testicle could not be isolated from this mass. No pathological inguinal nodes were detected.

Ultrasonography detected a pronounced increase in the volume of the right testicle, with a 5.6 cm cephalocaudal diameter, hipoecoic and diffusely heterogeneous; these aspects suggested an expansive lesion.

A Computed Tomography Scan of the pelvis and abdomen revealed a right testicle partially visualized with increased dimensions embedded in the context of a proliferative mass; the fat around the spermatic cord was prominent. Peri-testicular vascular ingurgitation was also present that seemed to have a connection with the splanchnic/mesenteric circulation.

Tumor markers for testicular cancer, β-HCG and α-FP, were within the normal range.

We assumed the patient had a testicular tumor and he was proposed to radical orchiectomy.

During the surgery, contrary to expectations, it was ascertained that inside the scrotum the patient had a healthy right testicle and spermatic cord together with an indirect inguinal hernia whose sac surrounded the large mass (Fig. 1 ).


Appearance of the structures inside the right scrotum of the patient during the ...


Figure 1.

Appearance of the structures inside the right scrotum of the patient during the surgery; inside the right scrotum the patient had a healthy right testicle (thin white arrow) and spermatic cord together with an indirect inguinal hernia which sac (thin black arrow) was surrounding the big mass (thick black arrow); this mass was linked to the epiploon and splanchnic/mesenteric circulation (thick white arrow).

The mass inside the herniary sac was completely separated from it and only fixed to a small portion of the omentum and vessels; this portion was ligated, the mass was completely removed and the indirect inguinal hernia was corrected.

This tumor measured 12 × 9.5 × 7.5 cm and had a firm to soft consistency. Other macroscopic features included a solid cut surface, grey, with a brown/hemorrhagic stipple, and a central cystic hemorrhagic area of about 5 cm.

Microscopically, the tumor was a mesenchymal, spindled hypocellular proliferation, with cytonuclear atypia sparse or absent. These cells were in a stromal matrix containing small to medium sized blood vessels, composed of thick, eosinophilic collagen fibers with an admixture of thinner and lighter collagen fibers. Necrosis or mitotic figures were absent (Fig. 2 ). Immunohistochemical analysis showed that the neoplastic cells reacted positively with vimentin (Fig. 3 ) and focally with desmin. Immunoreactivity was negative for: smooth muscle actin, CD117, B-catenin, ALK, CKAE1-AE3, CA5.2, Calretin, WT1, trombomodulin, S100, CD68, CD99, bcl2 and IgG4.


Photomicrograph of the tumor reveals a mesenchymal, spindled hypocellular ...


Figure 2.

Photomicrograph of the tumor reveals a mesenchymal, spindled hypocellular proliferation in a stromal matrix, containing small to medium size blood vessels, composed by thick, eosinophilic collagen fibers (white arrow) with an admixture of more thin and light collagen fibers (black arrow) (hematoxylin-eosin, ×100).


Immunohistochemical analysis showing that the neoplastic cells reacted ...


Figure 3.

Immunohistochemical analysis showing that the neoplastic cells reacted positively with vimentin. (vimentin, ×40).

The microscopic features and immunohistochemical profile were very suggestive that this mass could be an angiomyofibroblastoma-like tumor of the male genital tract.

After two years of follow-up, there was no evidence of recurrence .

Discussion

Fletcher et al.1 in 1992 described a new type of mesenchymal tumor named angiomyofibroblastoma that arose in the vulva and was different from aggressive angiomyxoma, which was locally invasive.2

According to the authors1 the designation angiomyofibroblastoma was based on the two integral components of this tumor: the blood vessels and the stroma; this tumor is composed of relatively bland spindled appearing cells and a loosely collagenous stroma with numerous small and medium sized vessels.3 ; 4 ; 5 ; 6  ;  7 Vessels are usually rounded but occasionally compressed, with perivascular fibrosis; intralesional fat may be present.7 ; 8  ;  9 There are differences between the clinical forms of these tumors in men and women.

Concerning immunohistochemical analysis, angiomyofibroblastoma reacts positively and diffusely with vimentin1 ; 5 ; 6 ; 7 ; 9  ;  10 and has variable expression of desmin,1 ; 4 ; 5 ; 6 ; 7 ; 9  ;  10 CD34,1 ; 3 ; 4 ; 5 ; 6 ; 7  ;  8 smooth muscle actin5 ; 7 ; 8  ;  10 estrogen and progesterone receptors.5 ; 7 ; 8  ;  9

The histogenesis of angiomyofibroblastoma is a subject of speculation,3  ;  9 but Laskin et al. consider that derivation of this tumor from a perivascular mesenchymal cell, with a capacity for fatty and myofibroblastic differentiation under environmental influences, cannot be excluded.7  ;  9 This progenitor cell could be related to the CD-34 positive fibroblast-like cells that normally exist around the vessels. Theoretically, this cell may lose its ability to express CD-34 with myofibroblastic differentiation,7 in male angiomyofibroblastoma.

Angiomyofibroblastoma-like tumor is a rare mesenchymal tumor that occurs in men in the scrotum,3 ; 4 ; 7 ; 8  ;  10 inguinal region,3 ; 4 ; 7 ; 8  ;  10 and perineum.3 This tumor was first described in 1997 as a cellular angiofibroma; its name was changed to angiomyofibroblastoma-like tumor by Laskin et al. in 19987 due to its similarities with the angiomyofibroblastoma tumor described in women in 1992.3  ;  8

Angiomyofibroblastoma-like tumor is a superficial, well marginated and indolent tumor,3 ; 6 ; 7 ; 8  ;  10 ranging in size from to 14 cm.7  ;  10 It is usually asymptomatic but is occasionally associated with pain.3 ; 5 ; 7 ; 8 ; 9  ;  10 The majority of cases described occur during the fifth to eight decades.3 ; 4  ;  6

In our case study we have an indolent and superfi 12 cm tumor that appears in the scrotum, all characteristics of an angiofibroblastoma-like tumor; but, instead of arising from the subcutaneous tissues as usual, it seems to have developed from an atypical structure for this type of tumor, the omentum and mesenteric/splanchnic circulation, the only structures linked to the mass; it was identifi in the scrotum only because it was herniated through an indirect inguinal hernia.

Mesenchymal tumors are described as having a relationship with inguinal hernias,2  ;  6 Some authors believe that the angiomyofibroblastoma-like tumor, could clinically simulate a hernia or a hydrocele,7 ; 9  ;  10 but in the available literature we do not find a description of this type of tumor herniated to the scrotum or linked to the omentum.

The recommended treatment for these tumors is surgical excision.3 ; 6 ; 8  ;  10 The lesion is usually non recurrent,1 ; 8 ; 9  ;  10 with only one case of recurrence described thirteen years after surgery.7

In conclusion, we describe an angiomyofibroblastoma-like tumor with a different clinical presentation and probably a different origin compared to the cases described before; in this case report the tumor had a subcutaneous presentation simulating a testicular tumor but apparently did not arise from the subcutaneous tissues of the scrotum, perineum or inguinal region as usual3 ; 4 ; 7 ; 8  ;  10 but from the omentum and the vessels of the mesenteric circulation, the only structures linked to the mass.

Ethical disclosures

Protection of human and animal subjects. The authors declare that no experiments were performed on humans or animals for this study.

Right to privacy and informed consent. The authors declare that no patient data appear in this article. The authors declare that no patient data appear in this article.

Right to privacy and informed consent. The authors declare that no patient data appear in this article.The authors declare that no patient data appear in this article.

Conflicts of interest

The authors have no conflicts of interest to declare.

References

  1. 1 C.D.M. Fletcher, W.Y.W. Tsang, C. Fisher, K.C. Lee, J.K.C. Chan; Angiomyofibroblastoma of the vulva. A benign neoplasm distinct from aggressive angiomyxoma; Am J Surg Pathol., 16 (1992), pp. 373–382
  2. 2 R.J. Clatch, W.K. Drake, J.G. Gonzalez; Agressive angiomyxoma in man. A report of two cases associated with inguinal hernias; Arch Pathol Lab Med., 117 (1993), pp. 911–913
  3. 3 M. Maruyama, T. Yoshizako, H. Kitagaki, A. Araki, M. Igawa; Magnetic resonance imaging features of angiomyofibroblastomalike tumor of the scrotum with pathologic correlates; Clin Imaging., 36 (2012), pp. 632–635
  4. 4 K. Miyajima, S. Hasegawa, Y. Oda, et al.; Angiomyofibroblastomalike tumor (cellular angiofibroma) in the male inguinal region; Radiat Med., 25 (2007), pp. 173–177
  5. 5 A. Blel, A. Mekni, M. Bel Haj Salah, et al.; Paratesticular angiomyofibroblastoma: case report and review of the literature; Pathologica., 100 (2008), pp. 489–491
  6. 6 B.K. Canales, D. Weiland, N. Hoffman, et al.; Angiomyofibroblastomalike tumors (cellular angiofibroma); Int J Urol., 13 (2006), pp. 177–179
  7. 7 W.B. Laskin, J.F. Fetsch, F.K. Mostofi; Angiomyofibroblastomalike tumor of male genital tract. Analysis of 11 cases with comparison to female angiomyofibroblastoma and spindle cell lipoma; Am J Surg Pathol., 22 (1998), pp. 6–16
  8. 8 Y. Iwasa, C.D. Fletcher; Cellular angiofibroma: clinicopathologic and immunohistochemical analysis of 51 cases; Am J Surg Pathol., 28 (2004), pp. 1426–1435
  9. 9 W.B. Laskin, J.F. Fetsch, F.A. Tavassoli; Angiomyofibroblastoma of the female genital tract. Analysis of 17 cases including a lipomatous variant; Hum Pathol., 28 (1997), pp. 1046–1055
  10. 10 L.R.M.F. Souza, E.C. Filho, W.P. Braga, P.T.C. Martins, H.D. Nicola; Angiomyofibroblastoma-like tumor of the inguinal canal; J Ultrassound Med., 28 (2009), pp. 1269–1272
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