Small cell lung carcinoma associated with paraneoplastic limbic encephalitis.

Limbic encephalitis is an unusual presentation of paraneoplastic syndrome. We report a case of small cell lung carcinoma associated with limbic encephalitis. A 67-year-old man presented with convulsions, memory impairment, and neuropsychiatric disturbances as initial symptoms. Subsequently, small cell lung carcinoma was diagnosed by bronchoscopic biopsy. The cerebrospinal fluid studies, electroencephalography, and magnetic resonance imaging findings had distinctive features compatible with paraneoplastic limbic encephalitis. The neuropsychiatric symptoms improved significantly after six cycles of systemic chemotherapy and adjuvant radiotherapy, resulting in partial remission of the tumor. A follow-up computed tomography scan of the head showed no evidence of intracranial metastasis 7 months after the diagnosis of cancer. Limbic encephalitis may be an initial manifestation of lung cancer. Greater awareness for diagnosis and early treatment of the primary tumor offers the best chance for improvement in patients with lung cancer presenting with limbic encephalitis.     

Elevated CA125 level associated with Meigs' syndrome: case report and review of the literature

Abstract.   Morán-Mendoza A, Luna GA, Ruiz GC, Olvera AS, López Graniel CM, Rincón DG. Elevated CA125 level associated with Meigs' syndrome: case report and review of the literature. Int J Gynecol Cancer 2006; 16(Suppl. 1): 315–318.
Meigs' syndrome is the association of ovarian fibroma, pleural effusion, and ascites. Meigs' syndrome with marked elevation of CA125 is an unusual clinical condition reported in 27 cases in the literature. The patient was a 46-year-old woman with right pleural effusion, ascites, ovarian tumor, and CA125 level of 1808 U/mL. Tomography revealed ascites and bilobate pelvic tumor of approximately 25 cm. The diagnosis of advanced epithelial ovarian cancer was considered, and the patient was treated with chemotherapy. Three chemotherapy schemes were applied due to the total lack of response in tumor volume; however, CA125 decreased to 90 U/mL. Thus, surgery was performed with resection of 25 cm of the left ovarian tumor, with intact capsule and without implants; the result of histopathologic analysis was fibroma. Postoperative CA125 was 11 U/mL. Patients with elevated CA125 and ascites cytology positive for malignancy must be cautiously treated due to the possibility of false positives, even if the probability is low. Therefore, minimally invasive surgery for biopsy collection must be considered. Although the association between ovarian tumor, pleural effusion, ascites, and marked elevation of CA125 is highly indicative of epithelial ovarian cancer, Meigs' syndrome must be considered in the differential diagnosis.     

Ovarian hemangioma with elevated CA125 and ascites mimicking ovarian cancer

We report a case of a very rare tumor of the ovary with an unusual presentation; an ovarian hemangioma with massive ascites and elevated CA125. A 57-year-old woman presenting with elevated CA125, massive ascites and a left solid adnexal mass of 60 x 47 mm, with calcification and increased blood flow at Doppler examination, was submitted to laparotomy. Frozen section was inconclusive and a staging procedure which complicated the patient was performed. Pathologic examination revealed cavernous hemangioma which is an extremely rare tumor of the ovary. Although it is very unusual, an ovarian hemangioma may present with ascites and elevated CA125 and the differential diagnosis from ovarian cancer should be considered.     

Intra-abdominal desmoplastic small round cell tumor in a 68-year-old female

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) of the peritoneum typically occurs in young adults. The mean age of females with DSRCT is 20 years. We describe a DSRCT with an unusual age of presentation mimicking a metastatic ovarian neoplasm. CASE: A 68-year-old para 4 female presented with abdominal enlargement. Laparatomy showed multiple tumor nodules attached to the peritoneal surface. The tumor was debulked. The histological findings were characteristic for DSRCT. Adjuvantly the patient received cytotoxic chemotherapy but died of recurrent disease 3 months after initial diagnosis. CONCLUSION: DSRCT should be added to the differential diagnosis of unusual gynecologic malignancies in elderly as well as younger females. Identification of DSRCT is important because it can be confused with primary ovarian neoplasms.     

Small cell carcinoma of the cervix: a clinical study of 15 patients and review of the literature

Objective: Small cell carcinoma of the cervix is a very rare and aggressive tumor. Most gynecologic oncology centers have little experience with this tumor. The purpose of the study was to evaluate the University of Kansas’ experience with this tumor and compare findings with those found in current literature.Methods: Fifteen patients with small cell carcinoma of the cervix were treated at or in association with Kansas University Medical Center between 1977 and 1997. Clinical data including age of patient, pregnancy history, stage of tumor, recurrence, type of therapy, presenting symptoms, location of metastases, and overall survival were studied.Results: Age at diagnosis ranged from 20 to 83 years with a mean of 47. Two patients were nulliparous, 2 were primiparous, and 11 were multiparous. Five patients (33%) were stage I, 3 (20%) were stage II, 1 (7%) was stage III, and 6 (40%) were stage IV at diagnosis. Four patients (27%) had progressive courses without good response to treatment, 7 (47%) recurred at an average of 15 months. The patients were treated with surgery, radiation, chemotherapy, or a combination thereof. Extrapelvic metastases developed in 5 of 8 patients with stage I or stage II disease with distant nodes, liver, lung, and brain being common sites. Three patients (20%) developed brain metastases. Tumor lysis syndrome was encountered in one patient. One patient was alive and well 80 months after diagnosis and one patient was lost to follow-up. The remaining 13 died of their disease. Mean survival was 22.3 months for stage Ib, 40 months for stage II, 27 months for stage IIb, 8 months for stage III, and 19.2 months for stage IV.Conclusions: Small cell carcinoma of the cervix is a rare and aggressive variant of cervical cancer. Our experience with this tumor raises the question of increased incidence of central nervous system metastases with small cell carcinoma. Present therapy has not significantly improved outcome with this tumor. There is conflicting evidence regarding the etiologic role of an infectious agent. Tumor lysis syndrome is a possible risk when treating these patients.     

A case of pulmonary type of ovarian small cell carcinoma

Small cell carcinoma is a rare form of ovarian cancer with a poor prognosis. It is divided into two types, the hypercalcemic and the pulmonary type, of which the latter is extremely rare. A 49-year-old woman presented with an acute abdomen and was suspected to have torsion of a left ovarian tumor, which was followed up with an emergency operation. Postoperative pathological examination gave a diagnosis of the pulmonary type of ovarian small cell carcinoma. Six courses of paclitaxel and carboplatin therapy were given as adjuvant chemotherapy. The patient has survived for 36 months without recurrence. Here we present an extremely rare patient with the pulmonary type of ovarian small cell carcinoma.     

Krukenberg tumor of gastric origin in pregnancy with dismal outcome

Krukenberg tumors are mostly found as metastatic signet-ring cell adenomucinous carcinomas in young, premenopausal women. They are bilateral in 80% of the cases, and thus can be expected in pregnancy. A 31-year-old female was diagnosed by explorative laparotomy at 27 weeks of gestation with a Krukenberg tumor due to bilateral adnexal masses and a large amount of ascites. At surgery cesarean section with total abdominal hysterectomy, bilateral salpingo-oophorectomy, total omentectomy and pelvic lymphadenectomy was performed. The neonate died 24 hours later due to prematurity and respiratory distress syndrome. The primary site of the cancer was detected metachronously two months after surgery and postoperative chemotherapy, as stomach adenomucinous carcinoma. In spite of surgery and postoperative multiagent chemotherapy, the patient died six months from the diagnosis of Krukenberg.     

Erectile Dysfunction: Initial Symptom of a Patient with Lung Cancer

IntroductionPenile metastases are rare and represent the advanced stage of the primary tumor. The patients usually have a history of a previously diagnosed malignancy and when metastasis to penis occurs, the most common findings would be priapism, pain, and difficulty in voiding.AimWe aimed to present a patient who had erectile dysfunction as the initial symptom of lung cancer. Besides the unusual clinical presentation, the sonographic and magnetic resonance imaging (MRI) findings of the penile metastasis were also not typical.MethodsA 57‐year‐old man with erectile dysfunction was admitted to the Department of Urology. On physical examination, there was a rigid, smooth, immobile, and painless mass at the base of the corpora cavernosa. Ultrasonography and MRI were performed in order to delineate the nature of the lesion.ResultsRadiological findings could not lead to a certain diagnosis and the lesion could not be resected completely during the surgery. Therefore, biopsy of the corpus cavernosum penis was performed. The histopathological diagnosis was metastatic malignant epithelial tumor consistent with nonsmall cell carcinoma. Further investigations revealed a metastatic lung cancer.ConclusionsPenile metastasis may rarely be the initial presentation of a malignancy and erectile dysfunction may be a seldom symptom. Halioglu AH, Haliloglu N, Akpinar EE, and Ataoglu O. Erectile dysfunction: Initial symptom of a patient with lung cancer. J Sex Med **;**:**–**.     

Ovarian carcinoma associated with pregnancy. A review of 9 cases

BACKGROUND: The purpose of this study was to review patients with ovarian cancer in pregnancy, the effectiveness of the available methods of treatment and their prognosis. METHODS: A retrospective review of all women diagnosed to have cancer of the ovary associated with pregnancy who delivered at the authors' hospitals between January 1976 and December 2000. The demography, clinical presentation, time and mode of diagnosis, treatment, pregnancy outcome and maternal survival were noted. RESULTS: The incidence of ovarian carcinoma in pregnancy in the series was 0.08/1000 deliveries. Of the 9 patients, 7 had epithelial cancers; 4 serous cystadenocarcinoma, 2 mucinous cystadenocarcinomas and one undifferentiated cancer. One patient each had dysgerminoma and granulosa cell tumor. Six patients were in FIGO stage Ia, one Ic, one IIa. One patient was in stage III. Five patients were treated by unilateral salpingo-oophorectomy during pregnancy. Three patients had total abdominal hysterectomy, bilateral salpingo-oophorectomy and omentectomy followed by chemotherapy. Debulking of the tumor was done in a patient in stage III with subsequent chemotherapy. This patient died 13 months from the time of diagnosis of the tumor. The overall 5-year survival rate in the series was 78% and 100% for stage Ia. CONCLUSIONS: Association of ovarian cancer with pregnancy is a rare occurrence. Early diagnosis and appropriate treatment offers the best prognosis for the patient. The higher survival rates in the series was attributed to a larger number of patients in stage I of the disease and 2 patients with a germ cell tumor and dysgerminoma which have the best prognosis. Aggressive postoperative chemotherapy also contributed to the better outcome.     

Pseudo-Meigs syndrome and elevated CA125 associated with struma ovarii

BACKGROUND: Struma ovarii is a rare form of ovarian neoplasm composed entirely and predominantly of thyroid tissue. The association of pseudo-Meigs syndrome, elevation of CA125 and hyperthyroidism to struma ovarii is a rare condition. CASE: We report an unusual presentation of a postmenopausal woman with benign struma ovarii associated with pseudo-Meigs syndrome, hypertiroidism, and elevated CA125 serum level, and a large complex right pelvic mass thereby mimicking an ovarian cancer. CONCLUSIONS: Struma ovarii is a rare cause of ascites, hydrothorax, elevated CA125, and hyperthyroidism. This rare condition should be considered in the differential diagnosis in patients with ascites and pleural effusions but with negative cytology.     

A case of primary peritoneal serous papillary carcinoma initially presented by massive bilateral pleural effusions

Primary peritoneal serous papillary carcinoma (PSPC) is a rare primary peritoneal tumor. Clinically, PSPC usually presents with general abdominal discomfort resulting from variable amounts of ascites. In a state of small amounts of ascites, initial manifestation of massive bilateral pleural effusion is unusual. A 76-year-old female nonsmoker with no asbestos exposure complained of dyspnea during exercise. Chest radiograph showed a massive bilateral pleural effusion. Chest computed tomography (CT) revealed irregular pleural thickening and a small amount of ascites. Abdominopelvic CT revealed nodular thickening of the parietal peritoneum, mesenteric or omental nodules, omental cake, and lymphadenopathy in paraaortic regions. Adenocarcinoma cells were found via cytologic examination in bilateral pleural fluids and ascites. Because the primary site of the adenocarcinoma was not found, a surgical biopsy of the right pleural thickening was performed. The final diagnosis was PSPC. The patient was treated with platinum-based chemotherapy. Physicians should be aware of a possibility of PSPC when the radiographic findings show massive bilateral pleural effusion due to pleural carcinomatosis, with high serum levels of CA125.     

Rapidly progressing cervical cancer in a patient with human immunodeficiency virus infection

Many malignancies occur in association with the acquired immunodeficiency syndrome (AIDS). The incidence of cervical intraepithelial neoplasia is increased in patients with human immunodeficiency virus (HIV) infection, although coexistent HIV infection and cervical cancer have not been described. We describe a patient with HIV infection and a stage IIB, poorly differentiated cervical carcinoma who initially responded well to standard radiation therapy. Relapse at an unusual periclitoral site as well as disseminated carcinomatosis appeared within 2 months. Despite chemotherapy with cisplatin, bleomycin, and mitomycin C, the patient died within 3 months of relapse. This pattern of aggressive tumor behavior may occur more frequently as HIV infection spreads into the heterosexual population. We recommend frequent pelvic and cytologic examinations of HIV-infected women and the consideration of an aggressive treatment approach should invasive carcinoma be detected.     

Carcinoma of the uterine cervix with variegated histological patterns and calcitonin production

This is a report of a calcitonin-producing cervical cancer with variegated histological patterns. The levels of calcitonin correlated with the clinical course. Multiple lung metastases were completely controlled by cisplatin/Adriamycin/cyclophasphamide combination chemotherapy, although only for a short period. Microscopically, the tumor showed dominant small cell carcinoma; however, it did show differentiation into large cell nonkeratinizing carcinoma, keratinizing carcinoma, adenocarcinoma, and possibly carcinoid. Ultrastructural study revealed a few dense core secretory granules. Multiple immunohistochemical studies were performed. It is presumed that the tumor originated from the totipotent, immature precursor cells in the cervix and differentiated into endocrine carcinoma, squamous cell cancer, and adenocarcinoma.     

Mullerian adenosarcoma of the uterine cervix

The management of cervical adenosarcoma in a 14-year-old girl is described. The tumor had an exceptionally violent biological nature and did not respond to a variety of customary and unorthodox therapeutic measures. These included conventional intravenous chemotherapy, radiation therapy, surgery, intraarterial chemotherapy, colostomy, and peritoneovenous shunt for untreatable ascites. The patient died within 16 months of diagnosis. This is the fifth case of cervical adenosarcoma in the English literature. This tumor usually has a better prognosis and none of the previous four cases succumbed to the disease. The unusual virulence of the present case is discussed and the literature reviewed.     

Conventional and experimental prognostic factors in ovarian cancer

Epithelial ovarian cancer is the leading cause of death from gynaecologic malignancies in western countries. In the clinical day treatment decision of the physician (e. g. surgery, chemotherapy) based on individual prognostic factors of the patient with ovarian cancer. The tumor stage at time of diagnosis and the postoperative residual tumor mass are prognostic factors and are unequivocally related to overall survival. Other prognostic factors are identified mostly in small series and are discussed in the literature controversially. This article discussing the value of conventional prognostic factors, as stage, postoperative tumor mass, age, lymph node status, ascites) and newer molecular biological factors, as Her-2-status, PAI-1, MMP, VEGF and CD24.     

Acute respiratory distress syndrome caused by leukemic infiltration of the lung.

Respiratory distress syndrome resulting from leukemic pulmonary infiltrates is seldom diagnosed antemortem. Two 60- and 80-year-old women presented with general malaise, progressive shortness of breath, and hyperleukocytosis, which progressed to acute respiratory distress syndrome (ARDS) after admission. Acute leukemia with pulmonary infection was initially diagnosed, but subsequent examinations including open lung biopsy revealed leukemic pulmonary infiltrates without infection. In one case, the clinical condition and chest radiography improved initially after combination therapy with chemotherapy for leukemia and aggressive pulmonary support. However, new pulmonary infiltration on chest radiography and hypoxemia recurred, which was consistent with acute lysis pneumopathy. Despite aggressive treatment, both patients died due to rapidly deteriorating condition. Leukemic pulmonary involvement should be considered in acute leukemia patients with non-infectious diffusive lung infiltration, especially in acute leukemia with a high blast count.     

Postpartum Budd-Chiari syndrome with prolonged hypercoagulability state

Budd-Chiari syndrome after pregnancy is an extremely rare disease. Reported here is a case of postpartum Budd-Chiari syndrome with unusual features of prolonged hypercoagulability state. The disease occurred 2 weeks after delivery and despite massive anticoagulation treatment the patient developed severe hepatic vein occlusion, renal vein thrombosis, inferior vena cava thrombosis, and femoral artery thrombosis.     

Malignant transformation of an ovarian mature cystic teratoma presenting as a rectal mass

BACKGROUND: Squamous cell carcinoma arising from malignant degeneration of a mature cystic teratoma is rare with a reported incidence of approximately 1-3%. The most common presenting symptoms are lower abdominal pain and increasing abdominal girth of several months' duration. Approximately 50% of the patients present with FIGO stage I while 35-38% present with stage III diseases. CASE: The case described herein represents an unusual presentation and initial diagnostic dilemma of locally aggressive squamous cell carcinoma arising in an ovarian dermoid cyst, with invasion into the distal rectum and anal canal causing rectal bleeding similar to the presentation of anal squamous cell carcinoma. Despite aggressive surgical management with posterior exenteration and optimal tumor debulking followed by 5040-cGy pelvic radiation utilizing 25-MV photons, the patient developed pelvic recurrence at the vaginal cuff 6 weeks after completion of her adjuvant radiotherapy. She subsequently failed cis-platinum single-agent chemotherapy and died 9 months after her initial surgery and diagnosis. CONCLUSION: Squamous cell carcinoma in the anal canal, diagnosed by colonoscopy or proctoscopy, could be an unusual presentation of that arising from malignant degeneration of an ovarian dermoid cyst. This tumor may behave in a locally aggressive manner and be resistant to pelvic radiation or single-agent chemotherapy of cis-platinum. The current experience of adjuvant treatment after comprehensive staging and cytoreductive surgery reported in the world literature is limited, and the optimal management of the malignancy remains unclear.     

Ovarian carcinoma metastatic to bilateral axillary lymph nodes. A case report.

BACKGROUND: Ovarian cancer is a major cause of cancer deaths in women and usually presents with diffuse abdominal disease. Lymph node metastases are common, but axillary lymph nodes are rarely involved. CASE: A 63-year-old woman, initially presenting with abdominal symptoms and massive ascites, underwent optimal cytoreduction followed by intravenous chemotherapy with paclitaxel and carboplatin. The patient subsequently underwent a second-look surgery revealing only microscopically positive disease and then received intraperitoneal chemotherapy with cisplatin. At a follow-up visit, she was found to have bilateral axillary lymph node enlargement. Mammography revealed no lesions in either breast. Fine needle aspiration dissection confirmed the diagnosis of metastatic, recurrent ovarian adenocarcinoma and subsequently axillary lymph node dissection was undertaken. CONCLUSION: Ovarian carcinoma, which usually follows typical patterns of metastatic involvement, can appear in unusual areas.     

Desmoplastic small round cell tumor: report of a case presenting as a primary ovarian neoplasm

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is an intra-abdominal malignancy that typically has extensive peritoneal spread at the time of diagnosis. We report a case of DSRCT with involvement of the ovary and omentum as well as an elevated CA-125 level at presentation. CASE: A 23-year-old female presented to another institution with a pelvic mass and a CA-125 level of 140 U/ml. During tumor reductive surgery the right ovary, omentum, and liver were found to be involved. Initial histologic examination favored an undifferentiated small cell carcinoma of the ovary. The patient received two cycles of Taxol and cisplatin chemotherapy and was referred to the University of Texas M. D. Anderson Cancer Center. Upon review of the pathology material at the time of the referral, a diagnosis of DSRCT was made. Despite two additional cycles of chemotherapy, the tumor progressed, and the patient returned home. CONCLUSION: DSRCT may mimic an ovarian primary tumor by presenting with involvement of the ovary and an elevated CA-125 level, and should be included in the differential diagnosis of ovarian neoplasms in young patients.