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[Clinical significance of HNPCC, surgical aspects of early recognition].

A hereditary background may be demonstrated in approximately 15-20% of colorectal carcinomas. Familial adenomatous polyposis syndrome (FAP) constitutes about 1% of this patient population whereas hereditary non-polyposis colorectal carcinoma (HNPCC) makes up a further 3-6% of colorectal malignancies. The clinical features of HNPCC are dominant right colon localization, early age of onset, high prevalence of synchronous and metachronous tumors. Germline mutations of the so-called mismatch repair genes can be demonstrated in the genetic background of HNPCC. Screening and careful follow-up of these families are essential since the lifetime occurrence of colorectal carcinomas and HNPCC associated tumors has an 80-85% prevalence. The recognition of the affected families may be accomplished by taking a thorough family history, spanning several generations based on the Amsterdam and Bethesda Criteria, immunohistological investigations of the removed specimens and finally the exact identification of the pathologic MMR gene mutations. Radical surgical intervention is advised in cases of proven mutation carriers who are suffering from CRC. The index persons and their family members must be under regular control for their lifetime, with one-to-two year intervals to prevent fatal disease. The initiation of a national HNPCC register would further decrease the mortality and morbidity of the disease.

Colorectal Neoplasms, Hereditary Nonpolyposis↗

[One-stage operations on the lungs and other organs in multifocal and metastatic cancer].

Surgical treatment of 135 patients with multifocal and metastatic cancer of the lungs and other organs is analyzed. The absence of absolute differential diagnostic differences between true poly-neoplasia and synchronous or metachronous solitary metastases leads to extension of indications to surgical treatment of these patients. Surgical procedures were performed in 109 patients, 37 (33.9%) of them were one-stage. One-stage procedures meet the requirements of radical surgery and are most preferable biologically and clinically. These surgeries decrease treatment duration, prolong survival to 3-5 and more years in many patients. As cytoreductive operations, one-stage resections improve the condition of the patients, enable effective polychemotherapy.

Adrenal Gland Neoplasms↗

Expression of matrix metalloproteinase 7, laminin and type IV collagen-associated liver metastasis in human colorectal cancer: immunohistochemical approach.

BACKGROUND/AIMS: Matrix metalloproteinase 7 (MMP-7) plays an important role in vessel invasion and metastasis in human colorectal cancer. METHODOLOGY: The significance of MMP-7, laminin and type IV collagen expression in human colorectal cancer was investigated by immunohistochemical assay, and the correlation with liver metastasis was analyzed. RESULTS: In a synchronous metastasis group, 26 of 36 cases (72%) showed positive staining of MMP-7: There were 32 cases (89%) in the lymph channel and 28/32 cases (87%) in the vessels, and 17/34 cases (50%) showed a positive rate of laminin. In the metachronous metastasis group, 14 of 30 cases (47%) showed positive staining of MMP-7: There were 19 cases (63%) in the lymph channel and 13/19 cases (69%) in the vessels, and 17/30 cases (57%) showed a positive rate of laminin. In the control group, which was a 5-year disease-free group, despite there being no significant clinicopathological factors compared with the other two groups, 17 of 37 cases (51%) showed positive staining of MMP-7: There were 12 cases (37%) in the lymph channel and 6 cases (18%) in the vessels, and 2/31 cases (5%) showed a positive rate of laminin. The expression of type IV collagen attenuated in 19 out of 32 cases (59%) in Group S, 10 out of 19 cases (53%) in Group M, and 14 out of 37 cases (38%) in Group C, with no significant differences among the groups. Thus, the metastatic groups were significantly higher than the control group in terms of expression of laminin and MMP-7 in the lymph channel. CONCLUSIONS: These findings suggest that laminin and the expression of MMP-7 in the lymph channel is a useful parameter for predicting liver metastasis.

Aged↗

[Analysis of the clinicopathological features of Chinese hereditary nonpolyposis colorectal cancer].

OBJECTIVE: To investigate the clinicopathological features of Chinese hereditary nonpolyposis colorectal cancer (HNPCC). METHODS: Patients who met the Amsterdam criteria were enrolled in this study from several hospitals in China. Clinicopathological features of patients with HNPCC were compared between the patients with suspected HNPCC and sporadic colorectal cancer. RESULTS: One hundred and sixty-seven individuals from 31 families met the Amsterdam criteria. The average age was 48.6 (22-78) years old. There were 43 cases (31.9%) with ascending colon cancer and 52 cases (38.5%) with rectal cancer. The 3-, 5-, 10- years survival rate was 70.3%, 49.9% and 39.7% respectively. The incidence of multiple primary neoplasms was 20.4% . CONCLUSIONS: Chinese HNPCC is characterized by early disease onset. Rectal cancer and ascending colon cancer are the first and the secondly common cancer for Chinese HNPCC. Gastric cancer is the most common parenteral cancer in Chinese HNPCC families.

Adult↗

[Carney's triad: a short review apropos a case].

We communicate a case with the Carney triad (gastric leiomyosarcoma, pulmonary chondromatosis and extra-adrenal paraganglioma). It is, to our knowledge, the first case to be communicated in the Spanish scientific literature. We discuss some peculiar aspects of the debut and clinical evolution of this syndrome, together with its prognosis. We conclude that in clinical practice, the appearance in a young subject, specifically females, of multiple gastric myogenic tumors, should elicit the performance of further noninvasive procedures, needed to discard the diagnosis of the Carney triad.

Adult↗

Subsequent malignancies in patients treated with 131-iodine for thyroid cancer.

I-131 was administered to 298 patients with thyroid cancer, and there has been a follow-up of at least two years. Follow-up periods were: 2.5 to 30 years (median 14.5) in living patients, 2.5 to 15 years (median 5.5) in patients dead of tumour greater than or equal to 2 years after first treatment and 2.5 to 23 years (median nine) in patients dead without tumour. Person-years at risk were (total applied activity of I-131): 1119 (3 to 21 GBq), 1477 (22 to 65 GBq), 521 (61 to 170 GBq). 33 subsequent malignancies in 31 patients were observed, compared to an expected number of 17. The relative risk of subsequent malignancies is therefore 1.94 with a 95% confidence interval of 1.15 to 3.05. This increase in the incidence of subsequent malignancies after I-131 treatment is largely due to the significantly increased incidence of leukemia and bladder cancer. Estimated radiation doses to the bone marrow in the patients with leukemia were 301 cGy to 792 cGy and the doses to the bladder in patients with bladder cancer were 2250 cGy to 10, 350 cGy. After a total activity of less than 37 GBq I-131, no cases of bladder cancer or leukemia were observed. The observed number of subsequent malignancies are compared with the expected number according to several dose-effect estimations.

Adenocarcinoma↗

Multiple carcinoma in patients with psoriasis caused by psoralen-ultraviolet A therapy, natural killer cell insufficiency, or intensified sun exposure?

A case is presented of a patient with psoriasis in whom squamous cell carcinoma most likely occurred due to the interaction of various factors (psoralen-ultraviolet A therapy, sun exposure, and suppression of natural killer cell function). Therapy consisted of 1 mg/kg/daily of etretinate (Tegison), which resulted in clearing of psoriatic lesions in one month's time. New carcinomas did not develop and natural killer function improved.

Adult↗

[Solitary and multiple granular cell tumors. Case reports and review of the literature].

Two patients with multiple granular cell tumours (GZTs) of the skin and one patient with GZT of the tongue are reported. The tumours had been becoming larger for several years without subjective symptoms. Only the GZT of the tongue was diagnosed correctly before surgery; the cutaneous GZTs were identified only at histological examination. Benign GZTs reveal similar histomorphological features. In contrast, immunohistochemical studies yield conflicting results. Thus, cellular differentiation of GZT is still controversial. On current evidence, GZTs represent a distinct entity with a common histogeneis. The most widely accepted theory is that they originate from a cell in the peripheral nerve system, which has not yet been more precisely characterized.

Adult↗

Genital carcinoma after radiotherapy for carcinoma of the cervix. Report of two cases.

Two cases of genital carcinoma were found after radiotherapy for carcinoma of the cervix. Vulval carcinoma occurred 30 years after radiation with initial symptoms of itching and whitish changes of the skin at the external genitalia. Adenocarcinoma of the endometrium occurred 9 years after radiation and diffused intra-abdominal metastasis was found surgically. The sites of second malignancies following irradiation for cervical carcinoma and the time interval between them were reviewed. The characteristics of the post-radiation vulval carcinoma and the endometrial carcinoma were discussed.

Adenocarcinoma, Mucinous↗

Secondary neoplasms following cancer treatment with a special emphasis on lung tumors.

In patients with successfully treated Hodgkin's disease, lung cancers, and particularly small cell lung cancers, are not uncommon (relative risk: +/- 5) and result essentially from radiotherapy. What is usually considered as a late recurrence of small cell lung cancer occurs also frequently in long-term surviving patients after therapy for small cell lung cancer; it is hypothesized that at least some of these tumors might be in fact induced by the therapy used for the initial lung neoplasm.

Antineoplastic Agents↗

[Bladder carcinosarcoma with osteosarcomatous differentiation].

Osteoid tissue can eventually be identified outside the bone skeleton adopting the form of metastasis, osteoid metaplasia or primitive osteosarcoma. This latter presentation is most frequently located in soft tissues and exceptionally at the visceral level. The term used for any of these forms is extra-bone osteogenic sarcoma. This paper reports the case of a 62 year-old female patient diagnosed with vesical urothelial carcinosarcoma treated with external radiotherapy. After a disease-free period of 20 years the patient developed osteosarcomatous-differentiated vesical carcinosarcoma. A literature review is made to analyze, from a clinical and histopathological perspective, several items of interest presented by this pathology. The role of sarcomatous radio-induction is also highlighted. Finally, we emphasize the relevance of using radical treatments from the start, so as to restrain the highly malignant potential of this condition.

Carcinosarcoma↗

Benign granular cell tumor ("myoblastoma") of the stomach.

Two cases of benign granular cell tumor of the stomach which were found incidentally are reported. A review of the literature disclosed 10 case reports and three other cases which were mentioned. Despite the small size of the tumors eight of the 10 cases produced gastrointestinal signs or symptoms. A brief discussion of the histogenesis of the lesion is presented.

Carcinoma, Squamous Cell↗

Incidence and management of secondary malignancies in patients with retinoblastoma and Ewing's sarcoma.

Childhood cancer survivors at highest risk of developing a secondary malignancy are those with hereditary retinoblastoma. The majority of such secondary cancers will be sarcomas, most commonly of bone. One-third of these occur outside a typical radiation field, commonly in an extremity. Bone sarcoma is also the most commonly reported secondary cancer to develop among survivors of Ewing's sarcoma. In this group, radiation doses greater than 60 Gy as well as alkylating agent chemotherapy have been identified as contributors to the increased risk. The prognosis for patients with a secondary sarcoma has been poor, with few cures reported to date. However, an aggressive, combined modality approach, including radical resection, postoperative radiation, and adjuvant chemotherapy, may improve the survival rate.

Adolescent↗

[Angiosarcoma in a mastectomy scar following irradiation].

A 74-year-old patient who had undergone radical mastectomy and postoperative radiotherapy for invasive ductal mammary carcinoma developed an angiosarcoma in the radiotherapy field without associated lymphedema. The latency time was 178 months after the first and 82 months after the second radiotherapy. The total radiation-dose was 54 Gy. Only a few such cases have so far been described. These tumors are often multifocal and their prognosis is poor. Better known are angiosarcomas appearing after radical mastectomy as a consequence of chronic lymphedema, which are known as Stewart-Treves-syndrome.

Aged↗

[Spinocellular cancers and myxoid atypical fibroxanthoma of an actinically damaged burn scar].

We report on a 66-year-old male patient suffering from several squamous cell carcinomas and a myxoid atypical fibroxanthoma of the skin. The tumours developed in burn scars that the patient had had for more than 50 years and that had in part undergone actinic elastosis. The role of burn scars in the development of different skin tumours is pointed out. In addition, we discuss the differential diagnosis of the rare myxoid variant of atypical fibroxanthoma.

Aged↗