Malignant transformation of abdominal wall endometriosis to clear cell carcinoma: case report

ABSTRACT BACKGROUND: Malignant transformation of endometriosis in the abdominal wall is a rare and still poorly understood event. Less than 30 cases have been reported in the worldwide literature. Most cases of solid tumors are report in a previous abdominal scar with malignant transformation of a focus of endometriosis. Presence of lymph node metastases in nearby chains is frequent and is associated with poor prognosis. CASE REPORT: We report a case of a 42-year-old woman with a history of abdominal surgery (Pfannenstiel) to resect abdominal wall endometriosis. Physical examination revealed a solid mass of approximately 10 cm x 6 cm in the anterior wall of the abdomen. Computed tomography (CT) of the abdomen and pelvis showed a heterogeneous, predominantly hypoattenuating expansive formation measuring 10.6 cm x 4.7 cm x 8.3 cm. The patient underwent exploratory incisional laparotomy, block resection of the abdominal mass and lymphadenectomy of the external and inguinal iliac chains. The abdominal wall was reconstructed using a semi-absorbable tissue-separating screen to reconstitute the defect caused by resection of the tumor. Histological evaluation revealed infiltration by malignant epithelioid neoplasia, thus confirming the immunohistochemical profile of adenocarcinoma with clear cell components. Lymphadenectomy showed metastatic involvement of an external iliac chain lymph node. CONCLUSION: Resection of the mass along with the abdominal wall, with wall margins, is the most effective treatment. Reconstruction is a challenge for surgeons. The patient has been followed up postoperatively for eight months, without any evidence of disease to date.

Clear cell odontogenic carcinoma of the mandible: a treatment strategy

Abstract Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic tumor of the jaws, histologically characterized by the presence of agglomerates of cells with eosinophilic cytoplasm. The patient, a 62-year-old Caucasian woman, presented an intraosseous lesion in the mandibular symphysis. A clinical examination revealed a discrete volumetric increase with a hard consistency, palpable to extraoral and intraoral examinations. Imaging studies revealed an extensive radiolucent area, without defined limits, extending from the region of the right second premolar to the left canine. Incisional biopsy analysis indicated a diagnosis of CCOC. The treatment proposed was segmental resection of the mandible with a safety margin. After six months without recurrence, definitive mandibular reconstruction was performed using an iliac crest graft, followed by rehabilitation with implant-supported denture after five months. After three years of post-resection follow-up, the patient has shown no evidence of recurrence or metastasis. She continues to be under follow-up. To conclude, CCOC must be considered a malignant tumor with aggressive behavior. Previous studies have shown that resection with free margins is a treatment with a lower rate of recurrence. Nevertheless, long-term follow-up is necessary for such patients.

An analysis of current treatment practice in uterine papillary serous and clear cell carcinoma at two high volume cancer centers / 부인종양

OBJECTIVE: Despite the rarity of uterine papillary serous carcinoma (UPSC) and uterine clear cell carcinoma (UCCC), they contribute disproportionately to endometrial cancer deaths. Sufficient clinical information regarding treatment and prognosis is lacking. The aim of this study is to evaluate treatment outcomes in a rare cancer cohort based on the experience at two tertiary care cancer centers. METHODS: Clinicopathologic data were retrospectively collected on 279 patients with UPSC and UCCC treated between 1995 to 2011. Mode of surgery, use of adjuvant treatment, and dissection of paraaoritc lymph nodes were evaluated for their association with overall survival (OS) and progression-free survival (PFS). RESULTS: 40.9% of patients presented with stage I disease, 6.8% of patients presented with stage II disease and 52.3% of patients presented with stages III and IV. Median follow-up was 31 months (range, 1 to 194 months). OS and PFS at 5 years were 63.0% and 51.9%, respectively. OS and PFS were not affected by mode of surgery (open vs. robotic approach; OS: hazard ratio [HR], 0.68; 95% confidence interval [CI], 0.28 to 1.62; PFS: HR, 0.78; 95% CI, 0.40 to 1.56). Adjuvant treatment was associated with improved OS in stages IB-II (HR, 0.14; 95% CI, 0.02 to 0.78; p=0.026) but not in stage IA disease. There was no difference in OS or PFS based on the performance of a paraaoritc lymph node dissection. CONCLUSION: Minimally invasive surgical staging appears a reasonable strategy for patients with non-bulky UPSC and UCCC and was not associated with diminished survival. Adjuvant treatment improved 5-year survival in stages IB-II disease.

The survival outcome and patterns of failure in node positive endometrial cancer patients treated with surgery and adjuvant radiotherapy with curative intent / 부인종양

OBJECTIVE: The purpose of this study was to evaluate the patterns of failure, overall survival (OS), disease-free survival (DFS) and factors influencing outcome in endometrial cancer patients who presented with metastatic lymph nodes and were treated with curative intent. METHODS: One hundred and twenty-six patients treated between January 1996 to December 2008 with surgery and adjuvant radiotherapy were identified from our service's prospective database. Radiotherapy consisted of 45 Gy in 1.8 Gy fractions to the whole pelvis. The involved nodal sites were boosted to a total dose of 50.4 to 54 Gy. RESULTS: The 5-year OS rate was 61% and the 5-year DFS rate was 59%. Grade 3 endometrioid, serous, and clear cell histologies and involvement of upper para-aortic nodes had lower OS and DFS. The number of positive nodes did not influence survival. Among the histological groups, serous histology had the worst survival. Among the 54 patients relapsed, only three (6%) failed exclusively in the pelvis and the rest of the 94% failed in extrapelvic nodal or distant sites. Patients with grade 3 endometrioid, serous and clear cell histologies did not influence pelvic failure but had significant extrapelvic failures (p<0.001). CONCLUSION: Majority of node positive endometrial cancer patients fail at extrapelvic sites. The most important factors influencing survival and extrapelvic failure are grade 3 endometrioid, clear cell and serous histologies and involvement of upper para-aortic nodes.

Carcinoma de células claras variedad no hialinizante de parótida: comunicación de dos casos con diferente grado de diferenciación / Non-hyalinizing clear cell carcinoma of the parotid: report of two cases with different grade of differentiation

El carcinoma de células claras es una neoplasia rara de bajo grado que se desarrolla casi exclusivamente en glándulas salivales menores humanas. El objetivo de esta comunicación es analizar las características estructurales, histoquímicas e inmunohistoquímicas (IHQ) de dos casos de carcinoma de células claras de parótida y realizar el diagnóstico diferencial con otros tumores salivales primarios y metastásicos que presentan células claras. Cortes de ambos tumores fueron procesados para H/E, tricrómicos de Masson y Dane, Azul de toluidina, Azul alciano, PAS y PAS/diastasa; marcaciones IHQ para citoqueratinas de bajo y alto peso molecular, ki67, HMB45, p63 y proteína S-100. El patrón estructural de estos tumores estaba determinado por nidos y cordones de células claras delimitados por un estroma no hialinizado. En un tumor se observó una neoplasia maligna a células claras con manifiesta anaplasia. La expresión de ki67 fue importante. El otro tumor estaba constituido por células claras monomorfas sin signos manifiestos de atipia y casi nula expresión de ki67. Con PAS se demostró la presencia de glucógeno y no se observó un desarrollo importante del estroma colágeno en ambas neoplasias. En ambos casos resultó francamente positiva la inmunomarcación para citoqueratinas de bajo y alto peso molecular. Por el contrario resultó negativa para HMB45 y p63. La proteína S-100 tuvo su expresión en células aisladas. Se concluye que los casos presentados son de localización poco común (parótida) según lo descripto en la bibliografía; corresponden a la variedad no hialinizante ya que no está presente un importante estroma colágeno hialinizado, y ambos tumores son primarios de glándulas salivales, de origen epitelial, no melanocítico ni hematopoyético (linfomas) como lo demuestran las marcaciones IHQ.

The clear cell carcinoma is a rare neoplam of low level grade that develops almost exclusively in human minor salivary glands. The objective of this present work is to analyze the structural, histochemical and inmunohistochemical (IHQ) characteristics of two cases of parotid clear cell carcinoma and make a differential diagnosis with other primary and metastatic salivary tumors that clear cells show. Serial cuts of both tumors were processed for H/E, Masson and Dane trichromes, Toluidine blue, Alcian blue, PAS and PAS/diastase; IHQ marking of both high-and low-molecular weight cytokeratins, ki67, HMB45, p63 and S-100 protein. The structural pattern of these tumors were determined by nests and cords of clear cells delimited by a stroma non hyaline. In a tumor a malign neoplasia was observed in clear cells with anaplasia. The ki67 expression was important. The other tumor was constitued of clear monomorph cells without manifestation signs of atypia and almost no ki67 expression. With PAS was confirmed the presence of glycogen and not observed an important development of the collagen stroma in both neoplasms. Both cases resulted very positive the immunostaining of both high and low molecular weight cytokeratins. It resulted negative for the HMB45 and p63. The protein S-100 has it´s expression in isolated cells. We conclude that: the presented cases are very uncommon localization (parotid) as described by the literature; it matches a non hyalinizing variety because a very important hyalinized collagen stroma is not present, both primary tumors of salivary glands, of epithelial origin, non melanocytic, renal or hematopoietic lymphomas which shows immunostaining IHQ.

Metástasis tiroidea de un carcinoma de células renales: a propósito de un caso / Thyroid meatastases of a renal cell carcinoma: a case report

El carcinoma de células renales de células claras, es impredecible con comportamiento agresivo dentro de los tumores malignos, metastatiza en pulmón, hígado, piel, hueso, ganglios linfáticos, cerebro, cavidad nasal, encía, laringe y tiroides, pudiendo ocurrir antes o después del descubrimiento primario. Femenina 71 años, antecedente diagnóstico carcinoma de células renales en 1996. Intervalo libre de enfermedad 11 años. En 2008 se encuentra a la palpación tumor en lóbulo derecho del tiroides de 4 cm, móvil. Eco tiroideo: nódulo de 3,5 cm x 3,3 cm derecho. Punción con aguja fina: hallazgos compatibles con neoplasiafolicular. Se realiza: tiroidectomía total: carcinoma de células claras metastásico en ambos lóbulos del tiroides. El tumor metastásico tiroideo de un carcinoma renal es una entidad muy poco frecuente y asintomática, encontrándose como hallazgo en la necropsia. Ante un paciente con historia de cirugía por tumor renal y presencia de nódulo tiroideo se debe sospechar de metástasis

Carcinoma of the renal cells of clear cells has one of most essential landlord’s behaviors within the malignant tumors, metastases in lymphatic lung, liver, skin, bone, ganglia, brain, nasal cavity, larynx and thyroid, being able to happen before as much as after the discovery of the primary tumor. Feminine 71 years that diagnostic antecedent presents displays carcinoma of renal cells in 1996, free interval of disease 11 years. June 2008, being to the palpation in neck tumor 4 cm, moving body. Echo: Nodule of 3.5 cm x 3.3 cm. Puncture: Compatible findings with follicular neoplasia. Realized: Total thyroidectomy: Carcinoma of clear cells metastatic both lobes of the thyroid. The metastases thyroid tumor of renal carcinoma is an organization frequent little asymptomatic, being like finding in the post-mortem examination. Despite before a patient with history of surgery by renal tumor and presence of thyroid nodule one is due to suspect metastasis

Clear cell adenocarcinoma of the male urethral tract.

We present a rare case of clear cell adenocarcinoma of the male bulbomembranous urethra. Mostly these tumors have been described in the female urethral tract with its possible origin from mullerian remnants, wolffian remnants or paraurethral glands. Histologically, these tumors have typically tubulocystic pattern comprising of hobnailed cells with clear glycogenated cytoplasm along with well-defined cytoplasmic membranes. This case is being presented due to its rarity, aggressive behavior and to discuss, trauma as its possible etiological factor

Hyalinizing clear cell carcinoma of the base of tongue: A distinct and rare entity.

Hyalinizing clear cell carcinoma (HCCC) of tongue is a rare neoplasm originating from minor salivary glands. We present a case of HCCC involving the base of tongue, in a 73-year-old male, clinically diagnosed as fibroma. Laser excison of the mass was done. Histopathological examination showed an infiltrating lesion composed predominantly of clear clear. The differential diagnosis included other salivary gland lesions having a clear cell component and metastatic clear cell renal carcinoma. lmmunohistochemistry was useful in ruling out these lesions exhibiting clear cell component from clear cell carcinoma. imaging studies revealed no lesion in either kidney. Since, HCCC has a better prognosis and the adequate treatment is wide excision, it needs to be differentiated from other carcinomas with clear cells. No further therapy was given to the patient. One year after the surgery, the patient is symptom free without local recurrence and on regular follow up.

Pathologic characteristics, type of treatment and follow up of patients with uterine cervical carcinoma referred to the Radiation Oncology Department, Cancer Institute, Imam Khomeini Hospital,Tehran, Iran, 1995-2001.

BACKGROUND: Carcinoma of the uterine cervix is the sixth most common malignant neoplasm in women worldwide. Early stage diagnosis increases the cure rate of disease. Radiotherapy with or without concurrent chemotherapy is one of the most effective treatment modalities. After radiotherapy, accurate and regular follow-up results in early diagnosis and effective treatment of recurrence. METHODS: In this retrospective study, we evaluated 346 cases of cervical carcinoma who have been treated with radiotherapy in the Radiation Oncology Department of the Cancer Institute of Imam Khomeini hospital from 1995 to 2001. RESULTS: Age of the study group ranged from 26 to 78 (mean=50.5, SD=11). 30.4 percent of patients were early stage and 69.6 percent had advanced stage of disease. Some 92.2 percent of cases were squamous cell carcinomas and adenocarcinomas made up the 6.4 percent . Radical radiotherapy was most frequent radiotherapy setting and adjuvant radiotherapy (post-op) was the second. Most of the patients (43.7 percent) were followed for a short time, and a considerable number did not return for follow-up. CONCLUSION: According to our results, patients do not pay enough attention to disease follow-up. An acceptable training plan, with emphasis on regular follow-up, is recommended.

Rol patogénico del gen supresor de tumores PTEN en cáncer ovárico asociado a endometriosis / Pathogenic role of PTEN tumor suppressor gene in ovarian cancer associated to endometriosis

Background: Endometrioid carcinoma and clear cell carcinoma of the ovary are associated to endometriosis. Somatic mutations of PTEN (10q23.3) are present in endometrial endometrioid carcinoma. Therefore, these mutations could be also present in ovarian tumors. Molecular studies show that solitary endometriotic cysts are monoclonal, have aneuploid DNA, have a loss of 9p,11q and 22q heterozygosity (LOH) and a higher cellular proliferation index of the epithelial component. Aim: To determine the cellular proliferation index using Ki 67, the immunohistochemical expression of PTEN and LOH in patients with ovarian endometriosis without atypia (EN), ovarian endometriosis with atypia (EA) and endometriosis with adjacent ovarian carcinoma (ET). Material and methods: Paraffin embedded samples of 37 endometrioid and clear cell carcinomas of the ovary (CC/CE), 15 solitary ovarian EN and 15 ovarian EA, were studied. Expression of Ki 67 and PTEN was measured by immunohistochemistry. LOH of 10q23.3 locus was measured by polymerase chain reaction. Results: Ki 67 was 5.5 and 2.3% in EA and EN, respectively (p <0.005). There was a histological correlation between EA and a higher cellular proliferation index. PTEN was negative in 5 of 15 EN, 9 of 15 EA and 30 of 37 CE/CC. There was a correlation between LOH and loss of PTEN protein in EN, EA and ET (60%). Conclusions: Negative expression on PTEN in EN; EA; ET and CE/CC is a manifestation of the inactivation of this gene. The mechanisms that cause this inactivation, must be elucidated.

Solitary delayed contralateral testicular metastasis from renal cell carcinoma.

We report a 60 year old male presenting with contralateral testicular metastasis 7 years following radical nephrectomy for renal cell carcinoma. Testicular metastases from renal cell carcinoma reported in literature are predominantly ipsilateral and invariably on the left side. Usually these are present simultaneously with the renal primary or precede the diagnosis of renal tumors. Delayed contralateral testicular metastatic has not been reported to the best of our knowledge. The case highlights the unique behaviour of renal cell carcinoma with an unusual site of recurrence. The clinical presentation, pathogenesis and management of this rare presentation along with review of recent literature are discussed.

Enfermedad renal quistica adquirida. Entidad polisindromatica causa de progresion no unmunológica de insuficiencia renal / Acquired renal cystic disease. Polysyndromatic entity as cause of non-immunological progression of renal failure

Se comunica un paciente con una glomerulonefritis rápidamente progresiva que después de inmunosupresión y hemodiálisis recuperó parcialmente la función renal. Ocho años más tarde una TAC demostró una enfermedad renal quística adquirida (ERQA), entidad caracterizada por el desarrollo de numerosos quistes en los riñones de pacientes con diferentes nefropatias crónicas que no tienen historia de enfermedad quística hereditaria. La ERQA puede cursar en forma asintomática o como ocurrió en este caso con diferentes complicaciones derivadas de los quistes tales como poliuria-polidipsia, quiste renal hemorrágico, hematoma retroperitoneal y carcinoma de riñon a células claras. En doce años de seguimiento se observó una declinación lenta de la función renal que podría atribuirse a la ERQA. Se sugiere que la ERQA puede convertirse en un factor de progresión no inmunológico que condicionaria pérdia de la función renal cuando se instala en pacientes con insuficiencia renal moderada.

Clear cell carcinoma arising in a Cesarean section scar endometriosis: a case report

Endometriosis of a surgical scar is rare and occurs mainly when a hysterectomy or Cesarean section was performed. We describe a 54-year-old woman with a large suprapubic mass as a definite case of a endomerioid carcinoma developing within the scar endometriosis following Cesarean section. Scar endometriosis, as well as endometriosis at other sites, can turn malignant. Endometrioid carcinoma is the most common histological pattern of malignant tumor arising in endometriosis. But clear cell carcinoma is very unusual. A case of primary clear cell carcinoma in endometriosis of a Cesarean section scar is described. To the best of our knowledge, this is the first documented case of endomerioid carcinoma developing within the scar endometriosis in Korea.

Surface epithelial tumours of the ovary.

Surface epithelial tumours (SET) constituted 65.7% of all the ovarian tumours. Benign tumours were 182 (71.9%), of low malignant potential (LMP) 11 (4.4%) and frank malignant 60 (23.7%). Maximum number of cases, 102 (40.3%) belonged to 3rd decade. Mean age for serous cystoma was 31.5% years as compared to 30.8 years for mucinous cystoma. The commonest presenting feature was the abdominal lump observed in 182 cases (71.9%) and pain in abdomen in 120 (47.4%).Serous cystomas were t he most frequent tumours and comprised of 32.21% of all the ovarian tumours or 46.01% of all the SET or 65.56% of all the cystic SET. Seventeen (11.7%) of serous tumours were bilateral. Mucinous cystomas constituted 14.55% of all the ovarian tumours or 30.8% of all the SET. These tumours were bulky (78.6%; 15 cm diameter) and multilocular (83.9%).Mucinous cystadenocarcinoma was the commonest malignant epithelial tum our (36.6%). Endometroid carcinoma comprised 3.65% of all the SET or 8.4% of all the ovarian malignancy. Squamous metaplasia was seen in one case whereas 2 cases were of mesodermal mixed tumour with heterologous element as rhabdomyosarcoma. Clear cell carcinoma, Brenner tumour and unclassified group constituted 0.79%, 1.18% and 1.58% of all SET respectively.