Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This is a case report of endometrial adenocarcinoma in a patient with Turner's syndrome who had received estrogen-progestogen therapy for 13 years. At age 22 she was treated for primary amenorrhea and absence of secondary sexual characteristics. Urinary gonadotropin assay exceeded 32 mouse units/24 hours and infantile external genitalia, uterine hypoplasia, and impalpable ovaries were found. Cytogenetic studies confirmed a 45XO karyotype. For 13 years she received stilbestrol cyclically for 21 days with ethisterone, 5 mg twice daily, added for Days 16-21 of each cycle. Secondary sexual characteristics developed. After 13 years anemia developed, the uterus was found to be the size of a 20-week pregnancy, and an endometrial polyp protruding through the cervix showed squamous metaplasia. Following hysterectomy, histologic studies showed well-differentiated adenocarcinoma of the endometrium with some squamous metaplasia and osteoid formation. Metastases were not found. Similar cases treated with stilbestrol alone have been reported. Genetic predisposition is possible with this patient's X chromosome abnormality. However, estrogen therapy is thought to be a causative factor. Those receiving such therapy should have periodic gynecological examinations with curettage even when progestogens are also being used.
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PMID:Endometrial carcinoma after cyclical oestrogen-progestogen therapy for Turner's syndrome. 113 7

There is a 100-fold increase in the incidence of carcinomas of the vulva and anus in renal transplant recipients compared with the general population. Anogenital (anus, perianal skin, and external genitalia of both sexes) carcinomas occurred in 65 of 2150 renal transplant recipients who presented with 2298 different types of malignancy. Two-thirds of the patients were female and one-third male. They were much younger than persons with similar tumors in the general population. The average age of the females at the time of diagnosis was 37 years (range, 20-64) and of the males, 45 years (range, 34-62). The neoplasms occurred late after transplantation, an average of 88 months (range, 9-215), compared with an average of 56 months (range, 1-225.5) for all other post-transplant malignancies. The lesions involved the vulva, penis, scrotum, anus, or perianal area. Two patients also had involvement of the urethral meatus. In several female patients, there was a "field effect" with multiple tumors of the squamous epithelium of the anogenital area, vagina, or uterine cervix. Lesions ranged from in situ carcinomas (in one-third of the cases) to those with invasion of adjacent organs and lymph node metastases. Treatment varied from local excisions to radical vulvectomy, abdominoperineal resection, or penile resection, sometimes combined with excision of the inguinal lymph nodes. In several patients, there was a previous history either of condyloma acuminatum or herpes genitalis, suggesting a possible viral etiology of these tumors.
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PMID:Cancers of the anogenital region in renal transplant recipients. Analysis of 65 cases. 352 88

Two dogs with metabolic epidermal necrosis had hyperkeratosis of the footpads accompanied by erythematous, erosive and crusting lesions affecting the muzzle, external genitalia, perineum and periocular regions. Histopathological examination of skin biopsies revealed a superficial hydropic dermatitis with marked parakeratosis. Both dogs had high plasma activities of alkaline phosphatase and alanine aminotransferase and high concentrations of glucose, and also a marked hypoaminoacidaemia. Despite these similarities, the cutaneous eruptions were associated with different underlying diseases. One dog had a pancreatic carcinoma which had metastasised widely; the primary tumour and the metastases showed glucagon immunoreactivity on immunocytochemical staining, and the dog's plasma glucagon concentration was markedly greater than that of control dogs. The other dog had diffuse hepatic disease; its plasma glucagon concentration was similar to that of control samples and cirrhosis was identified post mortem. Metabolic epidermal necrosis in dogs is a distinct cutaneous reaction pattern which may be associated with different underlying systemic diseases; however, the pathogenesis of the skin lesions remains unclear.
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PMID:Metabolic epidermal necrosis in two dogs with different underlying diseases. 763 36

Two cases of primary extragonadal germ cell tumor of retroperitoneal origin are reported. One was a 26-year-old man complaining of back pain. He had a large retroperitoneal tumor with lung, liver and supraclavicular lymph node metastases. He was referred to us after being treated for malignant lymphoma. The serum AFP, beta-subunit of human chorionic gonadotropin (hCG-beta), CEA and CA-19-9 were elevated. The retroperitoneal disease was treated surgically and with radiotherapy. The pathological diagnosis was that of embryonal carcinoma and teratoma. The lung, liver and supraclavicular lymph node metastases disappeared completely after two courses of cisplatin-based chemotherapy. While further chemotherapy was postponed due to myelosuppression, the disease relapsed and was resistant to subsequent therapy. The patient died twelve months after he first saw us. The second case was that of a 36-year-old man complaining of edematous legs and external genitalia. He had an extensive retroperitoneal tumor with multiple pulmonary metastases. The serum AFP level was high. Suspected of having an extragonadal germ cell tumor, he was referred to us promptly. Cisplatin-based chemotherapy coupled with resection of residual retroperitoneal and pulmonary disease resulted in complete remission. The pathological diagnosis was that of possible embryonal carcinoma. Further chemotherapy was given as scheduled, using granulocyte colony-stimulating factor. The patient has been in complete remission for two years. The chemotherapeutic regimen and surgical policy in the treatment of the two patients were essentially same. Early diagnosis, adequate initial therapy and the use of granulocyte colony-stimulating factor may be relevant to the favorable prognosis in the latter case.
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PMID:Extragonadal germ cell tumor of retroperitoneal origin: report of two cases. 812 21

Two juvenile granulosa cell tumors in the testes of two newborns are described. The lesion, though rare, should be considered whenever a cystic testicular mass is encountered in newborns or young infants. Orchidectomy is the only treatment required because local recurrence or metastases have never been observed. Karyotyping of the patient is essential when the external genitalia are abnormal.
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PMID:Juvenile granulosa cell tumor of the testis: report of two cases in newborns. 916 70

Twenty-nine cases of naturally occurring, transmissible venereal tumor were studied retrospectively. The external genitalia was the primary site of tumor involvement in 27 dogs, with the remaining two dogs having primary intranasal involvement. Extragenital tumor involvement was identified in six cases, including five cases with metastatic disease. Fifteen cases were treated effectively with radiation therapy alone. Radiation therapy also was effective in four cases that were resistant to chemotherapy. Four of five cases treated with at least four doses of vincristine as a solitary agent also achieved complete remissions. Transmissible venereal tumor remains a unique canine tumor that often is curable despite the development of extragenital primary lesions or metastasis.
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PMID:Transmissible venereal tumor: a retrospective study of 29 cases. 982 80

Myxoid leiomyosarcoma is an uncommon tumor which, although previously well described in the uterus, is recognized to a lesser extent at other sites. We describe 18 cases of soft tissue leiomyosarcoma in which myxoid stroma occupied >50% of the tissue examined. Patients ranged in age from 22 to 84 years old (median, 57.5 yrs) and female patients outnumbered male patients 14 to 4. Tumor locations included the limbs (6 cases), female external genitalia (4 cases), head and neck region (3 cases), chest (2 cases), nipple, paratesticular soft tissue, and perineum (one case each). The tumors had a grossly gelatinous appearance and adopted three major histologic architectures: fascicular, reticular/microcystic, and "myxofibrosarcoma-like." The tumor cells were predominantly spindled in all cases with typical features of smooth muscle differentiation; there was a mixture of spindle and epithelioid cells in one case. No cases with pure epithelioid cytology were seen. All tumors displayed immunoreactivity for smooth muscle markers (smooth muscle actin 16/17, desmin 8/18) and, in addition, four cases were positive for keratin CAM 5.2 and three for epithelial membrane antigen. The tumors had a tendency to be morphologically lower grade (9 tumors were grade I, 8 were grade II, and only 1 was grade III). Follow up was available in 13 patients with a duration of 8 months to 41 years (median, 39 mos), and revealed local recurrences (often repeated) in five cases and metastases in two cases. There were three tumor-related deaths, of which two were the result of uncontrolled local disease. The differential diagnosis of myxoid leiomyosarcoma is broad and encompasses both benign and malignant lesions. Accurate diagnosis is critical because therapies may differ widely for entities in the differential diagnosis of myxoid leiomyosarcoma.
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PMID:Myxoid leiomyosarcoma of soft tissue, an underrecognized variant. 1089 15

Extramammary Paget disease (EMPD) often develops in external genitalia. Paget cells can, however, adopt an invasive phenotype and metastasize to regional lymph nodes and beyond, leading to poor patient outcomes. Based on this clinical observation, multiple lymphadenopathy may represent an initial sign of EMPD. To address the potential significance of multiple lymph node swelling in EMPD, we report two patients with cutaneous primary EMPD who showed multiple lymphadenopathy as an initial sign during the clinical course of the disease as well as tumour metastasis. Significantly, marked lymphatic vessel growth was observed in regional lymph nodes that underwent massive tumour cell invasion. Therefore, nodal lymphangiogenesis may promote tumour cell invasion and metastasis to distant organs, including the lymph nodes, emphasizing the clinical relevance of multiple lymphadenopathy.
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PMID:Multiple lymphadenopathy as an initial sign of extramammary Paget disease. 2084 66

Penile and preputial tumours are not uncommon in the horse, but can cause discomfort and lead to serious complications. Several types of tumour of the male external genitalia have been described. The most common type is the squamous cell carcinoma (SCC), which is found mainly in older horses. Reports of a breed predilection for penile tumour formation are equivocal, but castration, coat colour, poor hygiene and various infectious agents have all been suggested to predispose to the development of some types of tumour (e.g. SCC, papilloma and melanoma). Careful assessment of the primary tumour is an important first step in the design of an optimal treatment protocol. Invasiveness, differentiation grade, tumour size and presence of metastases are all relevant to the decision to pursue additional diagnostic procedures or specific treatment options. To date, no standard protocol has been reported for the approach to penile tumours in the horse and treatments range from minimally invasive therapies (e.g. topical use of 5-fluorouracil) to radical surgical interventions (e.g. en bloc penile and preputial resection with penile retroversion). Completeness of removal of the neoplasm and therefore risk of recurrence is highly dependent on the type of therapy chosen. However, the size and histopathological features of the primary tumour are also important factors with respect to the likelihood of recurrence. This review describes the most common penile and preputial neoplasms in the horse, and outlines a standard protocol aimed at arriving at a specific diagnosis and tailoring the therapeutic approach accordingly.
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PMID:Penile and preputial tumours in the horse: literature review and proposal of a standardised approach. 2103 6

A patient with female pseudohermaphroditism is chromosomally and gonadally a female individual but has male or ambiguous external genitalia. In this paper, we report a 12-year-old Chinese girl who was diagnosed with female pseudohermaphroditism characterized by clitoridauxe, hirsutism, acne, hypertension, and karyotype 46 XX. Computed tomography scan revealed a huge left abdominal mass with distant metastases to bilateral lungs and a concomitant pelvic teratoma. Because the left abdominal mass was unresectable, the patient underwent a biopsy of the abdominal mass and a radical resection of the pelvic teratoma. Histopathology confirmed that the left abdominal mass was an adrenocortical carcinoma (ACC) and the pelvic teratoma was a mature cystic teratoma originating from the left ovary. After surgery, the patient received a transcatheter arterial chemoembolization of ACC, combined with 2 g mitotane daily for systemic treatment. It was a pity that she died 8 months later after diagnosis. So far, as we know, the simultaneous occurrence of pseudohermaphroditism, ACC, and ovarian teratomas has not been reported in the literatures before.
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PMID:A rare adolescent case of female pseudohermaphroditism with adrenocortical carcinoma and synchronous teratoma. 2352 7


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