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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A "collision" tumor between a serous papillary adenocarcinoma and a steroid cell tumor of the ovary is described. No similar combination has been reported in the literature. The steroid cell component secreted testosterone, resulted in considerable virilization of the patient, and appears to have preceded the carcinoma by several years. It remains problematical whether the androgenic milieu may have predisposed to the development of the second, malignant, tumor.
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PMID:Collision tumor: serous adenocarcinoma and steroid cell tumor of the ovary. 132 20

Adrenal cortical carcinoma is a rare and highly malignant tumor. This retrospective study includes 99 patients (57 males and 42 females) reported to The Cancer Registry of Norway during the 15 year period from 1970 to 1984. The age adjusted incidence was 1.5 per million per year. Eighty-one patients were diagnosed with adrenal cortical carcinoma when still alive. In 18 cases the diagnosis was first made at autopsy. Median age at diagnosis was 54 years (range 2-88 years) with a slight male predominance. Information about initial symptoms was available in 67 patients, of whom 26 patients (18 females and 8 males) presented with clinically functioning tumors, the Cushing syndrome and virilization being most frequently encountered. Only 28 (35%) of 81 patients diagnosed when still alive had tumors confined to the adrenals. Half of the patients had distant metastases, with lungs and liver being the most frequent sites. Sixty-one patients underwent surgery; 51 patients had a radical or debulking operation and 10 patients had laparotomy with tumor biopsy. After a complete follow-up for at least 6 years (range 6-21 years), only 9 patients were still alive. Early stage (Stage I and II) and curative resection had a significant impact on the outcome of this disease. To improve prognosis, early diagnosis and radical surgery, if feasible, are needed.
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PMID:Adrenal cortical carcinoma in Norway, 1970-1984. 141 34

We describe a 44-yr-old woman with a 12-yr history of clinical virilization and serum testosterone levels up to 28.1 nmol/L (normal range, 1-3.3 nmol/L) in whom repeated clinical evaluation and surgical procedures failed to reveal the source of androgen production. At the time the patient was referred to the Clinical Center of the NIH, an intrathoracic mass was seen on upper cuts of an abdominal computer-aided tomography scan, confirmed by computer-aided tomography scan and magnetic resonance imaging of the chest. A 6 x 5 x 3.5-cm mass, attached to the posterior pericardium, was removed by thoracotomy. Pathological examination revealed an adrenal cortical neoplasm of uncertain malignant potential that contained testosterone, 11-deoxycortisol, progesterone, and 17-hydroxyprogesterone. After the operation, the patient's serum testosterone levels decreased to the normal range. Ectopic adrenal cortical rests in the thorax and neoplasms arising from these rests are extremely rare, and we are not aware of a similar case previously reported. In women with virilization, radiological studies of the thorax as well as other reported sites of ectopic adrenal cortex should be performed if radiological studies of the abdomen and pelvis fail to locate the source of the neoplasm.
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PMID:Virilizing adrenal cortical neoplasm arising ectopically in the thorax. 146 58

Postmenopausal hyperandrogenism with overt clinical effects is rare and often related to ovarian stromal disorders. We present a clinicopathologic study of 4 cases. The patients (age range 41-75 years; mean 62 years) had evidence of hirsutism or frank virilization. Their serum testosterone was elevated with or without increases in their serum androstenedione and DHEA levels. There were two right-ovarian hilus cell tumors, one associated with left-ovarian stromal hyperplasia and the other with bilateral hyperthecosis and nodular hilus cell hyperplasia. The other tumor was a small corticomedullary stromal luteoma with bilateral hyperthecosis and nodular hilus cell hyperplasia. The fourth patient had bilateral hilus cell hyperplasia with mild cortical-stromal hyperplasia. All these patients had rapid normalization of androgen levels after surgery without recurrence after a 2- to 10-year follow-up.
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PMID:Postmenopausal hyperandrogenism of ovarian origin. A clinicopathologic study of four cases. 152 32

The case is presented of a young female with virilization signs and total circulating testosterone levels above 4 ng/ml, without a concomitant increase in cortisol, 17 OH-progesterone, DHEA-S, or androstenedion levels. On CT scan exam a tumoral mass in the left ovary was observed with polycystic characteristics similar to those observed in ovarian cystadenoma, inspite of the fact that most androgenic ovarian tumors are solid. The pathological study revealed an ovarian Sertoli-Leydig tumor associated to a reticular pattern with heterologous chondroid and mucinoid elements of cystadenoma.
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PMID:[Sertoli-Leydig-cell ovarian tumor with a retiform pattern and heterologous elements: a rare cause of virilization]. 158 16

An adrenal carcinosarcoma is reported in a 29-year-old female presenting with clinical signs of virilization. This is the first reported case of a functioning adrenal carcinosarcoma in the English language literature. The tumor measured 12.5 cm in greatest dimension, weighed 610 g, and consisted of large areas of typical adrenal cortical carcinoma that was, however, interspersed with multiple foci of sarcoma. Rhabdomyosarcomatous elements were identified and confirmed both immunohistochemically and ultrastructurally. After radical resection, the patient received adjuvant mitotane therapy but developed rapid local and metastatic recurrence. Systemic chemotherapy was unsuccessful, and the patient died 8 months after surgery.
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PMID:Adrenal carcinosarcoma presenting in a woman with clinical signs of virilization. A case report with immunohistochemical and ultrastructural findings. 159 39

Fifteen girls with severe hyperandrogenism were investigated by us during the last 6 years. Thirteen of these were cases of untreated congenital adrenal hyperplasia (CAH) and 2 were cases of tumoral (one sertoli leydig cell tumor of the ovary and one adrenal adenoma) hyperandrogenism. Here we present the clinical profile and laboratory data of those with congenital adrenal hyperplasia. All the girls had masculinization of genitalia (clitoromegaly alone 5, clitoromegaly with varying degree of posterior labial fusion 8). Eleven cases had hirsutism and 9 had short stature. Two patients underwent unilateral adrenelectomy with diagnosis of adrenal adenoma. Hormonal profile confirmed the diagnosis of CAH with 21 hydroxylase deficiency (elevated 17 OHP levels with exaggerated 17 OHP response to ACTH) in 12 cases and 3 beta hydroxy steroid dehydrogenase deficiency (elevated DHEAS and 17 pregnenelone levels and exaggerated DHEAS and 17 pregnenelone response to ACTH) in one case.
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PMID:Congenital adrenal hyperplasia among peripubertal girls with hyperandrogenism. 160 2

Apparent signs of hormonal activity were observed in 65.3% of patients with hormone-producing ovarian tumors of different histology. Feminization was evident in 69.1% of cases of granulosa-stromal cell tumors: it was most often seen in patients with mixed theca-granulosa cell tumors (86.2%) but was least frequent in those with Brenner's tumor (22.2%). The occurrence of proliferative lesions of the uterus such as hyperplasia of the endometrium, cervical canal polyps and uterine myoma (81.8%) was higher in patients with granulosa-stromal cell tumors, too, the highest rate being observed for cases of mixed theca-granulosa cell tumors (93.1%) whereas the lowest--for patients with Brenner's tumor (27.8%). Various clinical signs of masculinization were most often seen in cases of androblastoma (79.2%) whereas the proliferative disorders in that group were rare (12.5%). As a result, the authors placed mixed theca-granulosa cell, theca cell, granulosa cell tumors and Brenner's tumor in the order of decreasing feminizing activity. In the granulosa-stromal cell tumor subgroup, masculinization was most often observed in granulosa cell tumor patients (12.1%).
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PMID:[Clinico-morphological signs of hormone-producing ovarian tumors]. 166 40

Six cases of adrenal cortical tumors are presented with a discussion of the clinical features and histological findings. Five of the 6 children, aged between 6 mths and 6 yrs, presented with symptoms of hyperadrenalism, 4 with virilization and 1 with Cushingoid features. The remaining infant presented with an asymptomatic abdominal mass. In each case there was a unilateral tumor separated from the residual adrenal gland by a thin fibrous capsule. Surgical resection was the treatment employed and, in the 5 cases with functional tumors, perioperative hydrocortisone was given. None of the children received post-operative chemotherapy or radiotherapy. Bizarre cellular morphology, a high mitotic count and extensive necrosis were all seen in clinically benign disease, demonstrating not only the efficacy of local resection but also the difficulty in applying the usual histological criteria of malignancy to these pediatric adrenal tumors. At follow-up, 5 of the 6 patients are alive with no evidence of recurrent disease. The only death resulted from measles pneumonitis in the 1 child who presented with Cushing's syndrome.
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PMID:Adrenal cortical tumors in childhood--clinicopathological features of six cases. 174 73

Sclerosing stromal cell tumors of the ovary are an uncommon neoplasm that usually does not produce hormonal imbalances. Most patients showing a hormonal effect from this lesion have had menstrual cycle disturbances. Infertility and endometrial hyperplasia have also been described. One other reported case had masculinizing effects. Other authors have documented elevated levels of both estrogenic and androgenic hormones that corrected after surgery. A case of a pregnant 27-year-old Caucasian with hirsutism on her chin and neck and a male suprapubic hair pattern is presented. Elevated androstenedione, dehydroepiandrosterone, and free testosterone levels were present. A 3-cm left ovarian mass was excised and identified as a sclerosing stromal tumor. The histologic features included a pseudolobular pattern with focal areas of sclerosis and a two-cell population of spindled and polygonal cells. Immunohistochemical studies showed a positive vimentin reaction, weakly positive desmin and muscle-specific actin stains, and a negative cytokeratin stain. Following surgery the hormone levels returned to normal and the hirsutism resolved. A normal female infant without evidence of masculinization was delivered from the patient at term.
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PMID:Masculinizing sclerosing stromal tumor of the ovary during pregnancy. 175


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