Case Study: Urachal Carcinoma in a Young Man

Case Study: Urachal Carcinoma in a Young Man A rare case of urachal carcinoma in a young man Young Kwon Hong, Haeyoun Kang1, Moon Hyung Kang, Dong Soo Park INTRODUCTION Urachal carcinoma accounts for less than 1% of bladder cancers and approximately 40% of vesical adenocarcinomas. Historically, patients tend to have a poor prognosis, with 5-year survival rates of 6.5–61.0%. Therefore it is important to detect the disease as early as possible. Most urachal cancers have been reported at the middle or old age, and it is hard to think that urachal remnant has malignant change in a young man at the age of twenties. Here we introduce a very rare urachal cancer case in a young man and we would like to remind that urachal remnants have potential to change from benign to malignant as earlier than expected. CASE REPORT A 26 year old man visited our clinic with gross hematuria starting a few months ago. Urinalysis showed many RBC, and CT scan showed a 4.0 x 6.8 cm sized lobulated cystic mass over the bladder dome which was located midline to the right side. The mass has calcified thick wall with suspicious contrast enhancement (Fig 1). Cystoscopy showed a ball-shape extrinsic mass from the bladder dome with intact bladder mucosa (Fig 2). With an impression of urachal cancer laparoscopic surgery was performed. Camera port was placed 5cm above the umbilicus, and two instrument ports were placed 7cm lateral and 2cm caudal to the camera port. Anterior peritoneum was dissected deep and widely from the level of umbilicus down to the UB including median umbilical ligament and part of both medial umbilical ligaments. Cystoscope was introduced from the urethra into the bladder to illuminate the margin of the mass and to facilitate keeping enough distance from the mass during laparoscopic partial cystectomy. Bladder was repaired continuously with 4-0 vicryl suture. After placing a closed suction drain the mass was wrapped into an Endo CatchTM bag and squeezed out through the extended umbilicus opening. A Foley catheter was inserted into the bladder and the port sites were closed. Cross section of the surgical specimen showed a unilocular cyst without definite solid part, filled with gray tan to brownish thick mucus material. It also showed whitish to yellowish thick wall with multifocal calcifications (Fig 3). Immunohistochemical staining was strong positive on CEA, positive on CD15 (LeuMI), and weak positive on CK7 and CK20. Final diagnosis was well differentiated mucinous urachal adenocarcinoma invading bladder muscle, pT3a based on Sheldon’s staging system, pT2b based on Mayo system, and pT2 based on Ontario system. Surgical margin was negative. DISCUSSION Malignant involvement of the urachus is rarely reported, one in 5 million people, accounting for 0.35% to 0.7% of all bladder cancers [1]. It has been reported mostly in adults over 30 years old. To our best knowledge, only one adult case of urachal carcinoma diagnosed before the age of 26 has been reported in the English written literature, although two cases have been reported in 15 year-old girls [2-7]. The youngest adult case in a 22 year-old patient is found on MD Anderson cancer center experience written by Siefker-Radtke et al [4]. According to the MSKCC study of 24 cases, two young men who was 26, 29 years old each found to have urachal cancer [5]. The 26 year-old man received extended partial cystectomy, umbilectomy and pelvic LN dissection, and survived at only 0.9 month of follow up without long-term survival data. The 29-year-old man received extended partial cystectomy and umbilectomy, and survived at 17 months of follow up without local recurrence or metastasis. Even in a large series study, Mayo clinic study of 49 cases for 53 years, the youngest was 43 years old [8]. Our case is an urachal adenocarcinoma found in a 26 year-old man, the second youngest adult case ever reported. Age at the diagnosis of urachal carcinoma is important to understand pathogenetic transitional mechanism from benign to malignancy. Also clinicians should be aware that urachal carcinoma may occur early in the life time at the age of twenties. References Sheldon CA,Clayman RV,Gonzalez R,Williams RD,Fraley EE. Malignanturachallesions. J Urol.1984; 131(1):1-8. Cornil C,Reynolds CT,Kickham CJ. Carcinoma of the urachus. J Urol.1967; 98(1):93-5. Gupta S,Bhaijee F,Harmon EP. Mucinousneoplasmarisingin aurachalcyst: a first in the pediatric population. Urology.2014; 83(2):455-6. Siefker-Radtke AO,Gee J,Shen Y,Wen S,Daliani D,Millikan RE,Pisters LL. Multimodalitymanagement ofurachalcarcinoma: the M. D. Anderson Cancer Center experience. J Urol.2003; 169(4):1295-8. Gopalan A,Sharp DS,Fine SW,Tickoo SK,Herr HW,Reuter VE,Olgac S. Urachalcarcinoma: aclinicopathologicanalysisof24caseswith outcome correlation. Am J Surg Pathol.2009; 33(5):659-68. Pinthus JH,Haddad R,Trachtenberg J,Holowaty E,Bowler J,Herzenberg AM,Jewett M,Fleshner NE. Populationbasedsurvivaldataonurachaltumors. J Urol.2006; 175(6):2042-7; discussion 2047. Henly DR1,Farrow GM,Zincke H. Urachal cancer: role of conservative surgery. Urology.1993; 42(6):635-9. Molina JR,Quevedo JF,Furth AF,Richardson RL,Zincke H,Burch PA. Predictorsofsurvivalfromurachal cancer: a Mayo Clinic study of 49 cases. Cancer.2007; 110(11):2434-40. Acute Tonsillitis Chronic Ulcerative Colitis | Case Study Acute Tonsillitis Chronic Ulcerative Colitis | Case Study Katharine Buzzbee is a pleasant young girl of 9 years old. She was brought to the health center with complaints of sore throat with difficulty swallowing. Her mother states that Katharine has had similar symptoms three separate times this year. The illnesses begin with irritation in the throat that progress to pain. The pain radiates to her ears and she generally develops a fever during the course of each attack. The patient also has a history of developing frequent colds where she experiences headaches, a stuffy nose and frequent sneezing. Her older brother and sister have both recently had the common cold. The patient is otherwise healthy with no major illnesses or injuries. Clinical manifestations: On examination, Katharines palatine tonsils are swollen, red and surrounded by exudate. She has a temperature of 101.1 °F and complains of a running nose and headache. She appears tired and isnt as energetic as in our previous encounters. Diagnosis: acute tonsillitis. Throat culture confirmed strep. bacterial infection. Etiology: Acute tonsillitis is generally caused by a viral infection, most commonly caused by common cold viruses such as: adenovirus, rhinovirus, influenza, coronavirus, respiratory syncytial virus. The Epstein-Barr virus, herpes simplex virus, cytomegalovirus and HIV occasionally manifest as tonsillitis. The second cause of acute tonsillitis is bacterial1 (about 30% of patients). The common bacterium is group A Beta hemolytic streptoccal. Other strains of bacteria are less common. The patient listed above has this type of infection. Pathogenesis: virus or bacteria attacks tonsil tissue exiting the response of innate immunity and adaptive immunity of T and B cells contained in the tonsils. The lymphocytes fight the infection, which leads to inflammation. As long as the lymphocytes and other cells can weaken the virus/bacteria the damage to the cells within the tonsils will remain reversible. Treatment: 10-day course of oral penicillin2, rest and hydration. Prognosis: symptoms should resolve during course of treatment. If frequent onset of tonsillitis continues tonsillectomy may be recommended. Inflammatory response The bacteria is ingested or inhaled and attaches to the palatine tonsils (injury). At the gross level the tonsils become swollen with erythema causing pain and difficulty completing normal tasks such as swallowing. In the study by Liljaa, Raisanenb and Stenfors3, the researchers studied bacterial and epithelial cells from palatine tonsils of nine patients with positive streptococcus pyogenes infection. The reported findings showed, S. pyogenes could be identified both in the mucous layer covering the tonsils and attached to the surface epithelial cells. Long chains of coccus-shaped bacteria could be seen encroaching on the epithelial cell borders. S. pyogenes can apparently penetrate the mucous barrier, attach to the epithelial cells, spread from cell to cell and possibly penetrate into the outermost layer of the epithelial cells. These events in turn provoke cytokine production and/or complement activation, which induce inflammatory reaction in the tonsillar tissue (activation). Dead bacteria and bacterial debris will remain at the site of the infection and must be removed (phagocytosis) from this area by neutrophils and macrophages. Once the bacterium has been removed through phagocytes the tonsillar tissue will resume its normal form through resolution (healing process). Swelling and pain will cease. Chronic inflammation: Ulcerative Collitis Case study Jedediah Jones is a 24-year-old student pursing a degree in chiropractic medicine. He presents today with abdominal pain and frequent diarrhea containing mucus and blood. The onset of these symptoms began 2 months ago, but Jedediah decided to seek treatment when his frequent bowel movements began to disrupt his studies. Patient states that on average he has approximately 8 bowel movements per day, most frequently occurring after a meal. His abdominal pain is most severe before a bowel movement, but remains constant at the pain level of 4 out of 10. Patient says pain is 6 out of 10 at its worse. Patient attributes the symptoms to his newly adopted diet mostly consisting of fast food. Until recently he lived at home where all his meals were homemade. Patient has experienced an increased level of stress since beginning his studies (6 months ago) and subsequently quit smoking cigarettes. Patient exercises regularly, but has experienced a decrease in energy levels since the onset of symptoms. Patient denies other illness or injuries and is currently not taking any medication. Patients twin brother was recently diagnosed with Crohns disease and his maternal grandfather died of colon cancer at 48 years old. Clinical manifestations: LLQ tender upon palpation and entire abdomen appears distended. Patient claims 8 bowel movements per day with a watery consistency containing mucus and blood. Conjuctiva and finger nail beds appear pale. Diagnosis: ulcerative colitis, confirmed by positive biopsy removed during sigmoidoscopy. Inflammation of bowel appeared to extend no further than the descending colon with most severe inflammation and ulceration in the sigmoid section. Stool cultures ruled out parasitic infection. Etiology: the cause of ulcerative colitis is unknown, but factors such as genetics7, psychological stress, smoking cessation and poor diet have been attributed to onset of disease. Pathogenesis: as stated in the pathology textbook7, Most investigators believe that [ulcerative colitis and Crohn disease] result from a combination of defects in host interactions with intestinal microbiota, intestinal epithelial dysfunction, and aberrant mucosal immune responses. Treatment: Sulfasalazine 2 pills by mouth, 3 times per day. à ¢Ã¢â€š ¬Ã‚ ¨Rowasa (mesalamine) rectal suspension enema, at bedtime until bleeding stops. Prognosis: continue medication until symptoms have resolved completely. Maintenance therapy will be recommended indefinitely to avoid relapse. Inflammatory response Podolsky8 explains in his article that inflammatory bowel disease (IBD) is believed to be the result of an ongoing activation of the mucosal immune system. This abnormal response is likely due to the defects in both the intestinal epithelium and the mucosal immune system (injury). Podolsky8 clearly defines the inflammatory response of IBD, Chronic, recurrent intestinal inflammation appears to result from stimulation of the mucosal immune system by-products of commensal bacteria in the lumen. Stimulation may occur as a result of the penetration of bacterial products through the mucosal barrier, leading to their direct interaction with immune cells, especially dendritic cells and lymphocyte populations (chemotaxis). Alternatively, bacterial products may stimulate the surface epithelium, possibly through receptors that are components of the innate immune-response system; the epithelium can, in turn, produce cytokines and chemokines that recruit and activate mucosal immune cells (transmigration). Cellular changes occur in ulcerative colitis as the chronic inflammation may lead to mucosal atrophy7, damage to the muscularis propria and disrupt neuromuscular function leading to colonic dilation and risk of perforation (cellular changes). As stated in the textbook7, The inflammatory process is diffuse and generally limited to the mucosa and superficial submucosa. Submucosal fibrosis, mucosal atrophy and distorted mucosal architecture remain a residual of healed disease but histology may also revert to near normal after prolonged remission (healing process). Resources Merck Manuals Online Medical Library. Tonsillopharyngitis. Available at: http://www.merck.com/mmpe/sec08/ch090/ch090i.html. Accessed: October 21, 2010 Venes D, Thomas C (Eds.). Tabers Cyclopedic Medical Dictionary 19th ed. Philadelphia, PA: F.A. Davis; 2001 Liljaa M, Raisanenb S, Stenfors L E. Initial events in the pathogenesis of acute tonsillitis caused by Streptococcus pyogenes. Int. J. Pediatr. Otorhinolaryngol. September 1998; 45 (1): 15 20. Mansson A, Adner M, Cardell L O. Toll-like receptors in cellular subsets of human tonsil T cells: altered expression during recurrent tonsillitis. Respir Res. 2006; 7(1): 36 Ebenfelt A, Ericson L E, Lundberg C. Acute Pharyngotonsillitis is an Infection Restricted to the Crypt and Surface Secretion. Acta Otolaryngol (Stockh) 1998; 118: 264 271. Wikipedia contributors. Wound healing. Wikipedia, The Free Encyclopedia. October 23, 2010, 14:00 UTC. Available at: http://en.wikipedia.org/w/index.php?title=Wound_healingoldid=392404843. Accessed October 24, 2010. Kumar V, Abbas A, Fausto N, Aster J. The Gastrointestinal Tract (Ch 17). Robbins and Cotran Pathologic Basis of Disease 8th ed. Philadelphia, PA: Saunders Elsevier; 2010: 810 814 Podolsky, D. Inflammatory Bowel Disease. N Engl J Med 2002; 347:417-429 The Empire Writes Back | Green is the Colour The Empire Writes Back | Green is the Colour Ashcroft, Bill et al in the book The Empire Writes Back: Theory and Practice in Post Colonial Literature discusses what post-colonial is and explains among others the theories of post-colonialism. According to Ashcroft et al, the term colonial can be used to cover all the culture affected by the imperial process from the moment of colonization to the present which concerns with the world as it exists during and after the period of European imperial domination and the effects of this on contemporary literatures. These contemporary literatures include those produced in the African countries, Australia, Bangladesh, Canada, Caribbean countries, India, Malaysia, Malta, New Zealand, Pakistan, Singapore, South Pacific countries, Sri Langka and even the USA (p.2). Development of post-colonial literatures happens through several stages along with the stages of national or regional consciousness and the project of asserting difference from the imperial centre (ibid, p.5). The first stage is the stage of the imperialism where writings were produced using the language of the imperial centre because they were written mainly by the literate elites which can also be regarded as the representative of the imperial power. Examples include gentrified settlers (Wentworths Australia), travellers and sightseers (Froudes Oceana, and his The English in the West Indies or the travel diaries of Mary Kingsley) or the Anglo-Indian and West African administrators, soldiers and boxwallah and their memsahibs (volumes of memoirs). At this stage the writers not only talk about the landscape, language and culture of the place but also emphasize the home over the native, the metropolitan over the provincial or colonial and so on (ibid, p.5). The second stage involved literature produced by what Aschroft et. al termed as under the imperial license by the natives or outcasts like the English educated Indian upper class or the African missionary literature. However, the institution of Literature in the colony was still under the control of the imperial ruling class which prevented the writers from fully exploring their anti-Imperial potential. One of the examples given is a novel entitled Ralph Rashleigh by James Tucker. Basically, there are four post colonial models which can be used to study literary texts. The first model is the national or regional models which emphasize the distinctive features of the particular national or regional culture; while the second one is the race- based model which identifies certain shared characteristics across various national literatures such as the common racial inheritance in literatures of the African diaspora addressed by the Black Writing model. Comparative models of varying complex ity which seek to account for particular linguistic, historical and cultural features across two or more post-colonial literature is the third model while the last one which is rather comprehensive is the comparative models which argues things such as hybridity and syncreticity as the constitutive elements of all post-colonial literature. One of the popular preoccupations of postcolonial literature deals with nationalism. Several definitions of nationalism can be found in the dictionaries and among them are the devotion to the interests or culture of ones nation; the belief that nations will benefit from acting independently rather than collectively emphasizing national rather than international goals; and aspirations for national independence in a country under foreign domination (http://www.thefreedictionary.com/nationalism). Benedict Anderson (1983) defines nation as imagined, limited and sovereign communities. According to Anderson imagined community is different from an actual community because it is not (and, for practical reasons, cannot be) based on everyday face-to-face interaction between its members. Anderson argues that nations are a small idea shared by a geographically limited area of people that despite its limitation has influence and power in its greater region and sovereignty over its people. Nation became more and more realized as linguistic diversity, religious authority and traditional monarchies faded. A nation is imagined because the members of even the smallest nation will never know most of their fellow-members, meet them, or even hear of them, yet in the minds of each, lives the image of their communion and regardless of the actual inequality and exploitation that may prevail in each, the nation is always conceived as a deep, horizontal comradeship. Members of the community probably will never know each of the other members face to face; however, they may have similar interests or identify as part of the same nation. Anderson believes that his creation of imagined communities became possible because of print-capitalism. Capitalist entrepreneurs printed their books and media in the vernacular (instead of exclusive script languages, such as Latin) in order to maximize circulation. As a result, readers speaking various local dialects became able to understand each other, a nd a common discourse emerged. Anderson argued that the first European nation-states were thus formed around their national print-languages. Quayum (2009) in his introductory note for Writing A Nation Essays on Malaysian Literature stated that nationalism implies idealisation of the nation that has many positive qualities in its ideal state. Besides creating the sense of belonging, solidarity and shared membership for all its citizens where all members recognise mutual rights and duties towards one another, it also creates a sense of conviction and loyalty among members to certain shared artefacts of the nation. Quayaum however believes that to some extent nationalism may also be dangerous because there is a probability that the idealisation of the nation can turn into idolatry and nationalist sentiment can deteriorate into demagoguery, divisiveness, despotism, radicalism and racism which may lead to sectarian arrogance and destroy the creative bond of the wholeness of humanity. Lloyd Fernandos Green is the Colour Lloyd Fernandos Scorpion Orchid (1976) and Green is the Colour (1993) provide crucial insights into the state of belonging and nation-formation, as well as the understated realities of racial religious segregation and politics of prejudice which are still rampant in the contemporary Malaysian ideological landscape (Qayum and Nam, 2009). Although the exact time frame for Lloyd Fernandos novel Green is the Colour has not been clearly stated, it is quite possible that the historical context which Fernando is indirectly referring to was the Malaysian Racial Riot of 13th May, 1969. The novel exposed the unstable relationship between the racial and religious groups of Malaysia which continues to haunt the socio-cultural fabric of the country up until today (Ng, 2009). Thus, the depiction of violence, prejudices between different races and roaming vigilante in the countryside were quite real and became a common sight at that time. The effect of the racial unrest which is referred in the novel as the unsightly scab in Malaysian history can directly be felt by the multicultural citizens represented by characters like Siti Sara, Dahlan, Yun Ming and Gita. The ill-fated, cross-cultural relationship between Siti Sara and Yun Ming and their reflections to the uncertain situation in the country become the focus of the novel. Yun Ming, a second generation Chinese is a civil servant working for the Ministry of Home Affairs who seeks justice by working from within the government. He lusts for Sara, an oversea- graduate lecturer teaching in a local premier university from the first moment he sees her at a concert. Since the first meeting, Yun Ming seems to feel a kind of attraction towards Siti Sara and always tries to find ways or opportunities to be near her. Siti Sara who at first tries not to make her feeling obvious due to her status as Omars wife finally responds and begins to find comfort and peace in Yun Mings company who is described as gentle and moderate and looks at things from a human point of view and believes in the brotherhood of all. His sincerity and trustworthiness are evidenced through his willingness to risk his life in d istributing relief goods in the Malay areas even during the peak of racial riot. The understanding and tolerance that Sara never gets from her spouse leads Sara to be deeply in love with the Chinese guy. Despite all the challenges and disapproval from others (except from Lebai Hanafiah, Siti Saras father), the couple is almost successful in their relationship until Yun Ming is caught under the instruction of the villain in the novel- Panglima. Siti Sara is very significant in the novel not only as a heroine but also a narrator. As such, the story is mainly narrated from her viewpoint except for Chapter 9 which is narrated by Lebai Hanafiah, Siti Saras father and Chapter 15 by Dahlan a lawyer. Saras father has certainly becomes instrumental in shaping her into a liberal, tolerant and accommodative woman ( ). Although described as not having a unitary, parochial sense of identity like her lover Yun Ming, nor adopt a monolithic model of nationalism like her husband Omar ( ), Siti Saras character is used to realise the authors philosophy of fellowship and understanding among multi-racial Malaysia ( ). Fernando tries to promote a tolerant, selfless Bangsa Malaysia in this novel through the depiction of the characters of Yun Ming, Siti Sara and Lebai Hanafiah. Through the story, Fernando not only shows the effect of explosive and intractable racial riots on the entire nation but also on the band of closely linked individuals and how their relationships are affected by the event particularly by their contesting views of the nation. The interpretation of nation or imagined community according to him would be a community that is open- minded, receptive and tolerant towards each other just like the relationship of Siti Sara and Yun Ming. Even though Panglimas single set of values may be necessary as a reference, it is wise to allow for some freedoms for the multiracial society to practice what they believe to be right and appropriate without letting their contesting views affect their judgments and relationship with other. As Quayum, (2007) puts it, as long as we consider love as the base of all metaphysics and humanity and as the highest principle in life, we can never go wrong in self-refashioning ourselves as a nation or a society. Fernando enunciated his firmly-held belief in the integration of races and religions in his two novels Scorpion Orchid (1976) and Green is the Colour (1993) while deftly criticising communal and divisive politics which inevitably result in intolerance and destruction. These two novels provide an insight into the state of belonging and nation-formation as well as the understated realities of racial-religious segregation and politics which are regarded as sensitive but anyhow still relevant to be discussed up till now (Ng, 2009). Even though Fernando seems to rely on the incidents of the 1969 racial riot to construct the ideological backdrop of the novel, it is however inaccurate to generalize it as depicting the realities of the tensions. Instead, a careful reading will reveal that the novel is in fact depicting the ideological state of affairs in Malaysia of the 80s and 90s, a situation which Fernando views as dire to the nations health due to the communal, prejudicial agendas of cer tain political groups. Fernando tries to bring to attention the dangerous direction towards which the nation is heading, a direction that is increasingly forgetting its multicultural makeup in the move towards racial-religious homogeneity (ibid. p.118-119). Although some critics feel that the portrayal of women characters in both Fernandos novel Scorpion Orchid and Green is the Colour is rather bias Mohammad A.Quayum who reads Siti Sara as a metaphor of a nation argues that her rape by Panglima is especially significant: she is at the centre of the novel and associated with the countryside and the natural beauty of Malaysia from time to time. The authors worst fears about the future of Malaysia would be realised if corrupt politicians and bureaucrats like Panglima were allowed to remain at the helm and rule of the country (Shaping a New National Destiny with Dialogic, p. 170) The novel reveals what would happen to Malaysia if megalomaniac, bigoted and close-minded politicians were to come into power and threaten to divest other racial groups of their rights to religious and cultural affiliations (Andrew Ng, 2009, p.119) Fernando articulates the dangers of deploying religion for selfish, politically-invested ends echoes precisely the socio-political situation in Malaysia during this period. (Andrew Ng, 2009, p.119) Other character include Dahlan, a renegade Malay lawyer who persistently questions Malay supremacy and privileges resulting in him being rejected by his community (Andrew Ng, 2009, p.119) Racial harmony cannot be reinstated by force. Panglima who represents the corrupted leader of the country and Ustaz Bahaudin a leader of a religious extremist have their own way to forward their homogenising, monolithic agendas. Panglima constantly talks about an ominous strategy for nation-building. According to him in order to maintain the order in the country everybody must follow one way of life and has one way of doing things. Tuan Guru Bahaudin and the Panglima endorse the view that a nation comprising many religions cannot become united and only through a single faith at the (potentially violent) expense of others can function as the adhesive for a people that is otherwise racially segregated (Wilson in Andrew Ng, 2009, p.123)