Primary Signet Ring Cell Carcinoma of the Duodenum: Case Report

Introduction: Primary carcinoma of the duodenum is an infrequent malignancy; it represents 0.3-0.5% of all carcinomas of the GI tract and 33-45% of all carcinomas of the small intestine. Clinical case: A 57-year-old female patient with nonspecific GI symptoms and unintentional weight loss was diagnosed with diffuse, infiltrating, and ulcerated adenocarcinoma in the duodenum. Surgical management with duodenopancreatectomy was considered; however, it could not be performed due to deteriorating nutritional status. She later died due to abdominal sepsis. Conclusion: The clinical manifestations of adenocarcinoma of the duodenum are variable and nonspecific. It is a challenge to make an early diagnosis. We recommend considering the diagnostic possibility in patients with nausea, emesis, abdominal pain, and weight loss, which requires esophagogastroduodenoscopy and histological and immunohistochemical confirmation.

Introducción: el carcinoma primario de duodeno es una neoplasia maligna infrecuente; representa el 0,3%-0,5% de todos los carcinomas del tracto gastrointestinal y el 33%-45% de todos los carcinomas de intestino delgado. Caso clínico: paciente de sexo femenino de 57 años, con sintomatología gastrointestinal inespecífica y pérdida de peso no intencional a quien se le diagnosticó un adenocarcinoma difuso, infiltrante y ulcerado en el duodeno. Se planteó el manejo quirúrgico con duodenopancreatectomía; sin embargo, no pudo realizarse debido al deterioro del estado nutricional y posteriormente falleció como consecuencia de una sepsis abdominal. Conclusión: las manifestaciones clínicas del adenocarcinoma de duodeno son variables e inespecíficas, es un reto realizar un diagnóstico precoz. Se recomienda considerar la posibilidad diagnóstica en pacientes con síntomas de náuseas, emesis, dolor abdominal y pérdida de peso, que requiere la realización de esofagogastroduodenoscopia y confirmación histológica e inmunohistoquímica.

Diagnóstico fortuito de neoplasia mucinosa de apéndice establecido durante la cesárea / Incidental diagnosis of mucinous neoplasm of appendix established during caesarean section

Resumen ANTECEDENTES: Los tumores del apéndice son bastante excepcionales y el adenocarcinoma mucinoso es el más frecuente de estos tumores. Esta patología es aún más infrecuente en pacientes embarazadas. CASO CLÍNICO: Paciente de 43 años, en curso de las 38.1 semanas del primer embarazo. Antecedentes: diabetes gestacional y obesidad mórbida. Se hospitalizó para practicarle la cesárea de urgencia, indicada por restricción del crecimiento intrauterino, sin posibilidad de una conducta expectante. En la ecografía de control prenatal del primer trimestre se describió la existencia de una masa quística, tabicada, de aproximadamente 12 x 12 cm. En la resonancia magnética nuclear de abdomen se encontró una lesión quística en el flanco derecho, de probable origen de masa anexial derecha de 12 x 9 cm. El estudio histopatológico reportó el hallazgo de una neoplasia mucinosa de bajo grado, asociada con extravasación extramural de mucina del apéndice cecal y un pseudomixoma peritoneal. CONCLUSIÓN: El hallazgo de una neoplasia mucinosa del apéndice en una paciente embarazada es sumamente raro. Su diagnóstico supone un reto, sobre todo en quienes no tienen síntomas. Aunque hay padecimientos más frecuentes en las mujeres, como los tumores de ovario o la apendicitis, vale la pena tener en cuenta este cuadro clínico en futuras pacientes. Se requiere mayor investigación de esta neoplasia en embarazadas para optimizar los métodos diagnósticos y poder tratarla de manera oportuna y evitar dañar al feto o la madre.

Abstract BACKGROUND: Tumours of the appendix are quite rare, with mucinous adenocarcinoma being the most frequent of these tumours. This pathology is even rarer in pregnant patients. CLINICAL CASE: 43-year-old patient, in the course of 38.1 weeks of her first pregnancy. History: gestational diabetes and morbid obesity. She was hospitalised for emergency caesarean section, indicated for intrauterine growth restriction, with no possibility of expectant management. The first trimester prenatal ultrasound scan showed a cystic mass, septate, measuring approximately 12 x 12 cm. Magnetic resonance imaging of the abdomen revealed a cystic lesion in the right flank, probably caused by a right adnexal mass measuring 12 x 9 cm. Histopathological study reported the finding of a low-grade mucinous neoplasm associated with extramural extravasation of mucin from the cecal appendix and a peritoneal pseudomyxoma. CONCLUSION: The finding of a mucinous neoplasm of the appendix in a pregnant patient is extremely rare. It is challenging to diagnose, especially in those without symptoms. Although there are more common conditions in women, such as ovarian tumours or appendicitis, this condition is worth considering in future patients. Further investigation of this neoplasm in pregnant women is needed to optimise diagnostic methods to treat it in a timely manner and to avoid harm to the foetus or the mother.

Absceso del psoas ilíaco secundario a un tumor de colon izquierdo fistulizado al retroperitoneo: caso clínico y análisis de la literatura / Iliopsoas abscess resulting from penetrating left colon cancer to the retroperitoneum: case report and literature review / Abscesso do iliopsoas secundário a tumor de cólon esquerdo fistulizado para o retroperitônio: caso clínico e análise da literatura

Los abscesos del psoas ilíaco secundarios a un tumor de colon fistulizado son excepcionales y potencialmente graves. La mayoría son adenocarcinomas de tipo mucinoso. Su tratamiento es complejo ya que, para lograr una resección oncológica pretendidamente curativa, es necesario realizar una resección ampliada con mayor morbimortalidad. Presentamos el caso de una paciente con un adenocarcinoma mucinoso de colon izquierdo fistulizado al músculo ilíaco y la pared anterolateral del abdomen en la que se realizó una resección multivisceral que incluyó el colon izquierdo, el músculo y la cresta ilíaca y parte de la pared anterolateral del abdomen.

Iliopsoas abscess secondary to perforation of colon cancer is an extremely rare and potentially life-threatening condition. Most tumors are mucinous adenocarcinomas. Its treatment its complex, as most patients need radical extended resections to achieve good oncological results, which are in turn, graved with higher morbidity and mortality. We present the case of a patient with a left colon mucinous adenocarcinoma penetrating to the iliopsoas muscle and the anterolateral abdominal wall that required a multivisceral resection including left colon, iliac muscle and crest and part of the anterolateral abdominal wall.

Abscessos do iliopsoas secundários a um tumor de cólon fistulizado são raros e potencialmente graves. A maioria são adenocarcinomas do tipo mucinoso. Seu tratamento é complexo, pois, para se obter uma ressecção oncológica supostamente curativa, é necessário realizar uma ressecção ampliada com maior morbimortalidade. Apresentamos o caso de um paciente com adenocarcinoma mucinoso de cólon esquerdo fistulizado para o músculo ilíaco e parede ântero-lateral do abdome no qual foi realizada ressecção multivisceral que incluiu cólon esquerdo, músculo e crista ilíaca e parte do a parede anterolateral do abdome.

Mucinous adenocarcinoma in perianal fistula in Crohn's disease: Case report and literature review.

INTRODUCTION AND IMPORTANCE: Crohn's disease (CD) is a chronic bowel disease that, due to exacerbated inflammation, can lead to complications such as the development of perianal fistulas. The development of mucinous adenocarcinoma in perianal fistulas in patients with CD is rare and, consequently, few reports exist in the literature. CASE PRESENTATION: We report the case of a 71-year-old man diagnosed 22 years ago with CD with perineal involvement, who came with complaints of intense perianal pain, a gluteal mass, and local bleeding. Tomography of his abdomen showed an expansive, heterogeneous, and solid perianal mass on the right, with interspersed necrotic/liquefied areas and possible mucinous content. The patient was referred to the surgery department for an incisional biopsy, which confirmed mucinous adenocarcinoma. The patient underwent extra levator abdominoperineal rectal resection (APR) with partial prostatectomy. CLINICAL DISCUSSION: Perineal mucinous adenocarcinoma arising in a fistula associated with CD is very rare. Since the symptoms overlap, early diagnosis of malignancy is difficult. Histological analysis is the gold standard for its diagnosis. Surgical resection through APR is well-established and, despite being a complex procedure with potential complications, tends to have good results. However, the locoregional and inguinal lymph node involvement was related to a worse progression in this case. CONCLUSION: The diagnostic hypothesis of mucinous adenocarcinoma should be suspected in CD patients who present long-term perineal involvement with fistulas. Biopsies and imaging exams should be performed to aid the diagnosis of the condition and thus contribute to the surgical plan.

Neoplasias apendiculares incidentales / Incidental appendicular neoplasms

Las neoplasias apendiculares se presentan hasta en el 50 % de los casos como un episodio de apendicitis aguda. Existen características demográficas, clínicas y radiológicas que aumentan las posibilidades de un tumor apendicular subyacente, sin embargo, en la mayoría de los casos, son los hallazgos intraoperatorios los que alertan al cirujano. A pesar de que el tipo histológico determina la radicalidad del manejo quirúrgico de estos pacientes, algunas características macroscópicas pueden orientar a las patologías específicas y a una conducta adecuada. En general, los objetivos del manejo quirúrgico inicial se cumplen con una resección limitada al apéndice cecal, asociada a la citología de mucina y biopsia de los implantes peritoneales si están presentes, reservando las resecciones extendidas, como hemicolectomía derecha oncológica, para los pacientes con compromiso extenso de la base o del mesenterio apendicular ante la sospecha de neoplasias neuroendocrinas o adenocarcinoma del apéndice cecal.

Appendicular neoplasms present in up to 50% of cases as an episode of acute appendicitis. There are demographic, clinical and radiological characteristics that increase the chances of an underlying appendicular tumor; however, in most cases are the intraoperative findings that alert the surgeon. Although the histological type determines the radical nature of the surgical management of these patients, some macroscopic characteristics can guide specific pathologies and appropriate behavior. In general, the objectives of initial surgical management are met with a limited resection of the cecal appendix, associated with mucin cytology and biopsy of peritoneal implants if present, reserving extended resections such as oncological right hemicolectomy for patients with extensive compromise of the base or appendicular mesentery when neuroendocrine neoplasms or adenocarcinoma of the cecal appendix are suspected.

Mucinous Adenocarcinoma With Intrapulmonary Metastasis Harboring KRAS and GNAS Mutations Arising in Congenital Pulmonary Airway Malformation.

OBJECTIVES: Mucinous adenocarcinoma arising in unresected congenital pulmonary airway malformation (CPAM) is rare. Underlying driver mutations in addition to KRAS gain-of-function mutations in this setting and the long-term outcomes of these patients are unknown. METHODS: We report a case of metastatic mucinous adenocarcinoma harboring both KRAS and GNAS mutations arising in a type 1 CPAM of a 14-year-old male. A literature review was performed. RESULTS: Next-generation sequencing revealed identical KRAS (G12V) mutations in both the CPAM and metastatic adenocarcinoma and a missense mutation in the GNAS (R201C) gene in the metastatic adenocarcinoma only. Median survival was 23 and 4 years for patients with localized (no or limited spread within the same lobe of CPAM) and distant involvement (spread to any different lobe of CPAM) of mucinous cells, respectively (95% confidence interval, 23-23 and 1.5-22 years, respectively; P = .017). CONCLUSIONS: Mucinous cell proliferation associated with type 1 CPAM has exceptionally good long-term outcomes if confined within the same lobe of CPAM. A second oncogenic mutation in the GNAS gene may be necessary for progression to malignancy and distant spread.

PET/MRI Characterization of Mucinous Versus Nonmucinous Components of Rectal Adenocarcinoma: A Comparison of Tumor Metabolism and Cellularity.

OBJECTIVE. The purpose of this study was to evaluate whether FDG PET/MRI can be used to differentiate the mucinous from the nonmucinous components of primary rectal tumors and to compare the glycolytic metabolism on PET with tumor cellularity on DWI in both components. SUBJECTS AND METHODS. Ninety-nine patients who underwent FDG PET/MRI for staging of primary rectal cancer were included in this prospective analysis. MRI depicted the mucin component through the tumor volume. Separate volumes of interest were drawn on both mucinous and nonmucinous components and propagated to PET and apparent diffusion coefficient (ADC) mapping. Maximum and mean standardized uptake values (SUVmax, SUVmean) and maximum, mean, and minimum ADC values (ADCmax, ADCmean, ADCmin) were recorded and compared between areas with mucinous and nonmucinous components. Whole-body PET/MRI was also used to evaluate for the presence of distant metastases. Nonparametric testing was used to compare the two groups of patients: those with tumors with a mucinous component and those with tumors without a mucinous component. Logistic regression analysis was performed to calculate the association risk between mucinous component and metastatic disease. RESULTS. Seventeen patients (17.2%) had a mucinous component within the tumor on T2-weighted MRI. Most of these patients had advanced disease, the mucinous component tumors being in significantly higher T categories than the tumors without a mucinous component (88.2% vs 61.0%; p = 0.032). SUVmax (7.4 vs 16.7; p = 0.002) and SUVmean (5.4 vs 13.4; p = 0.001) were significantly lower in tumors with a mucinous component than in those without a mucinous component. Tumor ADC measurements were not different between tumors with and those without a mucinous component (ADCmean, 1.4 vs 1.6; p = 0.361). There was no association between presence of a mucinous component within the primary rectal tumor and presence of synchronous metastases (odds ratio, 1.1 [0.4-3.0]; p = 0.904). Moreover, the occurrence of metastases in patients with mucinous component tumors (7/17 [41.2%]) was not different from that in patients with tumors without a mucinous component (28/82 [34.1%]) (p = 0.887). CONCLUSION. PET/MRI can be used to differentiate the mucinous and nonmucinous components within primary rectal adenocarcinoma on the basis of metabolic status. The FDG uptake is significantly lower in the mucinous component, but tumor cellularity based on MRI and DWI findings is not. Despite being associated with a higher T category in the sample of patients in this study, the presence of a mucinous component seems not to be associated with increased risk of synchronous metastases.

Adenocarcinoma mucinoso uracal / Urachal mucinous adenocarcinoma

Introducción: El uraco o conducto onfalomesentérico es una estructura embrionaria que conecta el intestino medio y el saco vitelino, que involuciona antes del tercer trimestre de embarazo. Objetivo: Presentar un caso con diagnóstico de adenocarcinoma uracal, neoplasia maligna rara. Desarrollo: Paciente de 50 años de edad sin antecedentes patológicos, con cuadro clínico inicial de ascitis y dolor abdominal, al examen físico masa palpable en hipocondrio-fosa iliaca izquierda que en la tomografía abdominal se observa una masa adyacente a la cúpula vesical y pared anterior del abdomen. El resultado de la biopsia reportó un adenocarcinoma mucinoso del conducto onfalomesentérico (uracal). Al momento del diagnóstico, el paciente se encontró en un estadio IVB según el sistema Sheldon y otros y un IV según sistema Mayo, etapa avanzada de la enfermedad que determina mal pronóstico. Se instauró tratamiento con quimioterapia neoadyuvante, quirúrgico y continuación con quimioterapia adyuvante, el cual continúa, actualmente, con una evolución estable. Conclusiones: Esta es una neoplasia rara. Sospechar su diagnóstico llevará a un diagnóstico temprano, lo que mejorará el pronóstico y sobrevida de los pacientes afectados(AU)

Introduction: The urachus or omphalomesenteric duct is an embryonic structure connecting the midgut and the yolk sac, which regresses before the third trimester of pregnancy. Objective: To report a case with a diagnosis of urachal adenocarcinoma, a rare malignant neoplasm. Case report: A 50-year-old patient with no pathological history, who had an initial clinical condition of ascites and abdominal pain. At physical examination, we found a palpable mass in the left hypochondrium - iliac fossa, which revealed a mass next to the bladder dome and anterior wall on the abdominal tomography of the abdomen. The biopsy result reported a mucinous adenocarcinoma of the omphalomesenteric duct (urachal). At the time of diagnosis, the patient was in stage IVB according to the Sheldon et al system and IV according to the Mayo system, an advanced stage of the disease that determines poor prognosis. Treatment with neoadjuvant and surgical chemotherapy was established and adjuvant chemotherapy to follow, which currently continues with a stable evolution. Conclusions: This is a rare neoplasm. Suspecting its diagnosis will lead to an early diagnosis, which will improve the prognosis and survival of affected patients(AU)

Mucinous adenocarcinoma of the appendix invading the urinary bladder.

Appendiceal carcinoma is a rare disorder. Although imaging exams can suggest carcinoma of the appendix simulating as a primary bladder cancer a transurethral biopsy is essential for diagnosis. We reported a case of a 27-year-old man, presented with hypogastric pain associated with recurrent gross hematuria and dysuria but without any intestinal symptoms such as pain, obstruction or melena. MRI revealed an enlarged appendix contiguous with the bladder. An en-bloc resection was performed and revealed appendiceal mucinous adenocarcinoma. Carcinoma of the appendix is an important differential diagnosis to other lesions and allow a good chance of cure by en bloc resection.

Adenocarcinoma mucinoso bien diferenciado de tipo gástrico (adenoma maligno): un reto diagnóstico / Gastric-type mucinous well differentiated adenocarcinoma (adenoma malignum): a diagnostic challenge

Resumen ANTECEDENTES: El adenocarcinoma mucinoso bien diferenciado de tipo gástrico (adenoma maligno) es una variante histológica poco frecuente de adenocarcinoma mucinoso cervical. La hidrorrea, el sangrado vaginal persistente y las formaciones quísticas cervicales son manifestaciones sugerentes de la enfermedad. CASO CLÍNICO: Paciente de 52 años, que acudió a consulta por hidrorrea y sangrado genital persistente. Después de la exploración física se tomaron muestras de: exudado vaginal, citología cervical, biopsia endometrial e identificación de virus del papiloma humano. También se practicaron estudios de: colposcopia, legrado endocervical, ecografía transvaginal, histeroscopia y resonancia magnética. Se visualizaron múltiples quistes de Naboth, menores de 2 cm; líquido en la cavidad uterina y el canal endocervical, sin hallazgos patológicos adicionales. Por la persistencia de los síntomas se decidió efectuar la histerectomía total simple con doble anexectomía, por laparotomía. El reporte anatomopatológico fue: adenocarcinoma mucinoso bien diferenciado de tipo gástrico, estadio IB2 (FIGO). Se prescribieron: tratamiento coadyuvante, radioterapia externa y braquiterapia. Después de 12 esquemas de tratamiento no se identificaron signos de recidiva. CONCLUSIONES: Establecer el diagnóstico anatomopatológico preoperatorio es decisivo para disminuir la morbilidad y mortalidad por adenocarcinoma mucinoso. Es importante establecer el diagnóstico diferencial exhaustivo con lesiones benignas cervicales.

Abstract BACKGROUND: Gastric-type mucinous well differentiated adenocarcinoma (adenoma malignum) is an infrequent cervical mucinous adenocarcinoma. It must be suspected if persistent hydrorrhea or vaginal bleeding are present, as well as multiple cervical cysts. CLINICAL CASE: A case of a 52-year-old woman with persistent hydrorrhea and vaginal bleeding. The study was completed with vaginal culture, cervical cytology and HPV (Human Papiloma Virus), colposcopy, endocervical curettage, transvaginal ultrasound, endometrial biopsy, hysteroscopy and magnetic resonance imaging. Multiple Nabothian cysts <2 cm and fluid in the uterine cavity and the endocervical canal were observed, with no other pathological findings. Due to the symptom persistence and severity, surgical treatment was decided, revealing the diagnosis of gastric-type mucinous well differentiated adenocarcinoma, stage IB2 (FIGO). Adjuvant treatment consisted in external radiotherapy and brachytherapy. After one year of follow-up, the patient did not show any sign of relapse. CONCLUSIONS: Reaching a pathological diagnosis prior to surgery has a crucial importance to decrease morbi-mortality. A thorough differential diagnosis including benign cervical lesions is essential.

Mucinous adenocarcinoma on perianal fistula. A rising entity?

INTRODUCTION: Mucinous adenocarcinoma on perianal fistula is a rare entity; it could be underdiagnosed because it behaves often as a regular perianal fistula. MATERIALS AND METHODS: We have recently treated four cases in our unit. We present them and review the literature, emphasizing on clinical characteristic and therapeutic options. The four patients were male with a mean age of 64. Three of them were classified as locally advances cases and therefore treated with neoadjuvant therapy. RESULTS: All of them underwent laparoscopic abdominoperineal escisión. Surgical specimens are described and clinical characteristic specified. Review of the literature shows that this disease has a very high potential risk of local recurrence and we must be aggressive with the resection. Sometimes plastic surgery is needed to reconstruct the perianal wound. CONCLUSIONS: Mucinous adenocarcinoma associated with anal fistula is a rare disease. Neoadjuvant chemoradiotherapy followed by an adequate abdominoperineal excision may result in favourable outcomes.

Prognosis of Signet Ring Cell Carcinoma of the Colon and Rectum and their Distinction of Mucinous Adenocarcinoma with Signet Ring Cells. A Comparative Study.

Signet ring cell carcinoma (SRCC) of the colorectum is very rare, comprising between <1% and 2.4% cases of colorectal cancer. Patients' prognoses are poor. Several case reports had described as SRCC cases that are mucinous adenocarcinomas (MAC) with signet ring cells (SRC). In order to clearly delineate between MAC with SRC and SRCC, we performed a retrospective study at a national cancer referral center in which survival and clinicopathological characteristics between these two forms were compared and also SRCC were characterized by immunohistochemistry. We retrieved 32 cases that had been classified as either SRCC or MAC with SRC subtypes. It was noted that SRCC patients presented at older ages, demonstrated more advanced clinical stages, lymphovascular invasion, lymph node metastases, and higher carcinoembrionic levels than MAC with SRC patients. Regarding SRCC immunophenotype, 50% showed loss of CDX2 expression, 33% were CK20 negative, 41.7% were CK7 positive, and 25% were negative for both CK7 and CK20. For the MAC with SRC and SRCC groups, the median disease-specific survival (DSS) was 46.1 months (95% CI 36.9-55.25) and 22.4 months (95% CI 5.1-39.7 [p = 0.039]), respectively. The 3-year DSS was 80.7% and 28.6% (p = 0.017) for the MAC and SRCC patients, respectively. Univariate and multivariate analyses showed that SRCC was associated with decreased survival. SRCC had several clinicopathological features that permitted differentiation of MAC with SRC from SRCC patients, who had a poor DSS. A differential diagnosis for metastatic gastric cancer is only possible with a good clinicopathological correlation.

Adenocarcinoma mucosecretante como complicación de la hidradenitis supurativa / Mucinous adenocarcinoma in association with hidradenitis suppurativa

El primer caso de adenocarcinoma mucosecretante asociado a una hidradenitis supurativa crónica, es presentado. La paciente posee una historia de 10 años de su hidradenitis; cuando esta se localiza en el área perineo-genital puede surgir un cáncer subyacente, siendo el carcinoma espinocelular el más frecuente. La enferma se sometió a una resección local amplia pero con un criterio paliativo. Se discute el tratamiento de la neoplasia, al igual que la incidencia de la misma bajo el tratamiento biológico, con adalimumab.

This is the first case in Argentina of Hidradenitis Suppurativa associated with a perianal mucinous carcinoma. Hidradenitis suppurativa is a common disorder, probably autoinflammatory in apocrine bearing skin. When the lesions are located in genital and perineal areas they may be associated with cancer, usually squamous cell carcinoma. We describe a case of a 46-year-woman who has a history of 10 years of hidradenitis suppurativa and developed a big mucinous adenocarcinoma. Treatment is discussed tough the gold standard is the resection abdominoperineal. Our case was treated with a palliative criterion with a wide local surgery. Adalimumab is an accepted treatment for the chronic inflammation in the hidradenitis suppurativa; we do not believe this drug cause or worse the outcome of the malignant neoplasia.

Mucinous adenocarcinoma associated with chronic suppurative hidradenitis: Report of a case and review of the literature.

INTRODUCTION: Chronic suppurative hidradenitis (CSH) is a benign condition that can affect the perineal region and often leads to the formation of abscesses and fistulas. It is rare for CSH to undergo malignant degeneration into mucinous adenocarcinoma. PRESENTATION OF CASE: We report a case of a 55-year-old male patient with perineal CSH who suffered worsening long-term pain despite multiple surgical procedures to alleviate his symptoms. Pelvic magnetic resonance imaging (MRI) showed multiloculated cystic lesion on the left side wall of the distal rectum with gluteal extension. Pathological examination revealed mucinous adenocarcinoma. The patient underwent an abdominoperineal resection (APR) of the rectum with cutaneous muscle flap reconstruction. Although histopathological sections showed clear margins, the tumor recurred 6 months following surgery. DISCUSSION: Perineal mucinous adenocarcinoma arising in a patient with CSH is an extremely rare condition. This diagnosis is often difficult, due to the paucity of signs of malignant degeneration as well as the rarity of the disease itself. Surgical resection of the lesions is a well-established approach. In this case, diagnosing the tumor at such a late stage likely compromised his outcome. CONCLUSION: Malignant degeneration to mucinous adenocarcinoma must be suspected in patients with a history of long-term CSH. In such cases, local biopsies and a radiological examination, such as MRI can help in the diagnosis.

(18)F-FDG uptake by rectal cancer is similar in mucinous and nonmucinous histological subtypes.

PURPOSE: PET/CT has been considered limited for the evaluation of mucinous colorectal tumors due to low (18)F-FDG uptake. The aim of our study was to compare PET/CT variables in mucinous (MC) and nonmucinous (NMC) rectal adenocarcinomas. METHODS: Consecutive patients with cT2-4N0-2M0 rectal cancer included in a prospective clinical trial were reviewed. PET/CT was performed for primary baseline staging. Visual and quantitative analysis included SUVmax and SUVmean, metabolic tumor volume (MTV) and total lesion glycolysis (TLG). PET/CT parameters were compared according to histological subtypes. RESULTS: Overall, 73 patients were included (18 mucinous and 55 nonmucinous). SUVmax values were similar between MC and NMC (19.7 vs. 16.6; p = 0.5). MTV and TLG values were greater in the MC group (103.9 vs. 54.1; p = 0.007 and 892.5 vs. 358.8; p = 0.020) due to larger tumor volumes of MC. CONCLUSIONS: Metabolic parameters at baseline PET/CT for patients with rectal cancer are similar in mucinous and nonmucinous histological subtypes.

Solitary Splenic Metastasis from a Mucinous Adenocarcinoma of the Cecum. A Case Report.

Reports of cases of splenic metastasis are very rare, even more so when they are derived from a mucinous adenocarcinoma of the cecum. The most common presentation of a solitary splenic metastasis is from lung primary tumors, endometrium, ovary, cervix, stomach, colon, breast, bladder, and skin. We report the case of an 84-year-old woman with a solitary splenic metastasis from a mucinous adenocarcinoma of the cecum. Until this work, only 18 cases of solitary splenic metastases from colorectal carcinomas have been described in the literature.

[Video-assisted thoracic surgery diagnosis of mucinous (colloid) lung adenocarcinoma: Case report]. / Diagnóstico de adenocarcinoma mucinoso (coloide) pulmonar por cirugía torácica asistida por video. Reporte de caso.

BACKGROUND: Mucinous colloid lung adenocarcinoma is an uncommon variant of lung carcinomas with similar features to tumours seen in the gastrointestinal tract. To distinguish between these tumours and other mucinous lung tumours, such as mucinous bronchioloalveolar cell carcinomas and metastatic mucinous lesions could be difficult with small biopsy specimens from fine needle aspiration. CLINICAL CASE: The case is described of a 49-year-old female with history of dyspnoea and cough with bloody sputum and weight lose. Thorax axial computed tomography demonstrated a right lower lobe spiculated mass with calcifications. Transthoracic computed tomography- guided fine needle biopsy reported negative results, and the biopsy obtained with video-assisted thoracic surgery was useful for an adequate cytology report of a colloid variant of mucinous lung adenocarcinoma. CONCLUSION: Video-assisted thoracic surgery is an appropriate option for obtaining a larger specimen in those cases where small biopsies are inconclusive for the diagnosis of thoracic pathologies such as malignant tumours.

Mucinous adenocarcinoma arising from recurrent perianal fistula in patient with Crohn's disease: case report / Adenocarcinoma mucinoso surgindo de fístula perianal recidivante de paciente com doençde Crohn: relato de caso

Introduction: Anal carcinoma is a rare variant of epithelial tumors of the anal canal. When associated with chronic and active anal fistulas, usually this is an aggressive cancer that has difficult diagnosis and poor prognosis. Anal fistulas are a common manifestation of Crohn's disease (CD). This study aims to report a case of mucinous adenocarcinoma originating from recurrent perianal fistula in patients with CD. Case report: A man of 43 years, with melanoderma, complaining of perianal tumors, anal pain and mucopurulent secretion, the patient was diagnosed with fistulae. Colonoscopy revealed a chronic inflammatory process associated with villous polypoid lesion in the colonic and rectal mucosa. In a new episode, where it was diagnosed, chronic colitis of rectum and sigmoid was being prescribed sulfasalazine with improvement. There were relapses and the patient underwent repeated fistulectomias. After investigation, CD was diagnosed. Computed tomography (CT) of abdomen and pelvis showed multiple perineal and gluteal collections, and the patient underwent abdominoperineal resection of the rectum. Anatomopathological exam showed invasive mucinous adenocarcinoma. A new CT showed residual growth of the lesion. The patient was referred to the oncology referral service, where chemotherapy and radiotherapy were planned. The patient developed unfavorably, and his death occurred two months after treatment. (AU)

Introdução: Carcinoma anal é uma rara variante de tumores epiteliais do canal anal. Quando associado a fístulas anais crônicas e ativas, geralmente é um câncer agressivo que possui difícil diagnóstico e mau prognóstico. Fístulas anais são uma manifestação comum da doença de Crohn (DC). Este estudo tem como objetivo relatar um caso de adenocarcinoma mucinoso originado de fístula perianal recidivante em paciente com DC. Relato de caso: Homem de 43 anos, com melanoderma e queixas de tumorações na região perianal, dor anal e secreção mucopurulenta, sendo diagnosticada fístula. A colonoscopia evidenciou processo inflamatório crônico associado à lesão polipóide vilosa em mucosa colônica e retal. Em um novo episódio, constatou-se colite crônica em reto e sigmóide, sendo prescrito sulfassalazina com melhora. Houve recidiva do quadro e o paciente foi submetido a repetidas fistulectomias. Após investigação, diagnosticou-se DC. A tomografia computadorizada (TC) de abdome e pelve demonstrou múltiplas coleções perineais e glúteas, tendo sido realizada ressecção abdominoperineal do reto. O exame anátomo-patológico evidenciou adenocarcinoma mucinoso invasivo. Nova TC demonstrou crescimento da lesão residual. No serviço de referência oncológica, foram planejadas quimioterapia e radioterapia. O paciente evoluiu desfavoravelmente e veio a óbito após dois meses do tratamento. (AU)

GNAS is frequently mutated in both low-grade and high-grade disseminated appendiceal mucinous neoplasms but does not affect survival.

We analyzed a series of 55 disseminated appendiceal mucinous neoplasms treated at our institution for GNAS and KRAS mutations in an attempt to correlate mutation status with clinicopathological findings and patient survival. GNAS mutations (p.R201H, c.602G>A and p.R201C, and c.602C>T) were identified in 17 (31%) of 55 of disseminated mucinous neoplasms and were found in 8 (35%) of 23 low-grade mucinous neoplasms, 7 (37%) of 19 high-grade mucinous adenocarcinomas lacking a signet ring cell component, and 2 (15%) of 13 high-grade mucinous adenocarcinomas with a signet ring cell component. All 7 mucinous adenocarcinomas composed of pure (>95%) signet ring cells harbored wild-type GNAS. There was no significant association between GNAS mutations and sex and age (both with P > .05) or between GNAS mutations and individual adverse histologic features including cytologic grade, destructive invasion, tumor cellularity, angiolymphatic invasion, perineural invasion, and signet ring cells (all with P > .05). KRAS mutations were identified in 22 (40%) of 55 disseminated mucinous neoplasms. GNAS-mutated disseminated appendiceal mucinous neoplasms more frequently harbored concurrent KRAS mutations compared with GNAS wild-type tumors (65% versus 29%, P = .018). GNAS mutations were not significantly associated with overall survival (both with P > .05). Only overall tumor grade was an independent predictor of overall survival in the multivariate analysis (P = .01). Our results indicate that GNAS mutations are frequently identified in both low-grade and high-grade disseminated appendiceal mucinous neoplasms indicating that GNAS mutation status cannot be used to distinguish between low-grade from high-grade appendiceal mucinous neoplasms.