Gorham's syndrome: a case report and review of the literature.

Gorham's syndrome is a rare disorder involving a proliferation of vascular channels associated with extensive loss of bony matrix. A case report is presented with a review of the 97 previously reported cases. The age of patients at presentation has ranged from less than one to 75 years (mean: 27 years). Sixty-four percent have been men. Fifty-seven percent have had a history of prior trauma. Laboratory values for systemic measures have usually been normal. The disease usually arrests spontaneously, but this is unpredictable. Sixteen patients (16 percent) have died of the disorder, with 10 deaths due to chest wall involvement, three to spinal cord transection, two to sepsis, and one to asphyxia and aspiration. Although the mechanism of bone loss is unknown, osteoclasts were focally increased in the case described herein. Further information and investigation are needed to better understand this unusual disorder.

Peritoneal serous micropapillomatosis of low malignant potential (serous borderline tumors of the peritoneum). A clinicopathologic study of 17 cases.

Primary peritoneal serous micropapillomatosis of low malignant potential, or serous borderline tumor of the peritoneum, is a relatively rare lesion that is histologically indistinguishable from peritoneal "implants" associated with ovarian papillary serous tumors of low malignant potential. We analyzed 17 cases to further define the pathologic features and prognosis of this entity. The ages of the patients ranged from 16 to 67 years (mean, 33 years). Eight patients were symptomatic with chronic pelvic or abdominal pain (five patients), adnexal mass (one patient), small-bowel obstruction (one patient), and possible endometriosis (one patient). In nine cases (53%), peritoneal serous micropapillomatosis of low malignant potential was an incidental finding discovered during evaluation or treatment of other conditions. Grossly, the peritoneal lesions were focal or diffuse. They commonly appeared as miliary granules and often were believed to be peritoneal carcinomatosis. Microscopically, peritoneal serous micropapillomatosis of low malignant potential had all of the patterns seen in superficial ("noninvasive") peritoneal implants of ovarian serous borderline tumors. Psammoma bodies were a prominent feature of all cases. Twelve patients also had typical endosalpingiosis. Most patients were treated by hysterectomy and bilateral salpingo-oophorectomy. Surgical treatment in seven patients consisted only of biopsy. Ten patients had residual unresected disease at the time of their initial operation. Several patients received adjuvant chemotherapy. Follow-up was available for 14 of the 17 patients. One patient died of metastatic breast carcinoma at 3.8 years; another patient died 7 weeks after operation, possibly as a complication of therapy. The other 12 patients were alive at last known contact after follow-up intervals of 8 months to 16.2 years (mean, 7.5 years). Two of these 12 patients developed multiple episodes of small-bowel obstruction due to persistent peritoneal serous micropapillomatosis of low malignant potential; neither received adjuvant chemo- or radiotherapy. Both were alive without progressive disease 10.9 and 16.2 years after initial diagnosis, respectively. This excellent prognosis supports a regimen of conservative therapy for these patients.

Apoptotic bodies: a consistent morphologic feature of endocervical adenocarcinoma in situ.

To evaluate the occurrence of apoptotic bodies in endocervical adenocarcinoma in situ (AIS) and investigate the relationship of apoptosis to mitotic activity, we performed counts of apoptotic bodies and mitotic figures in 43 patients with AIS and in a comparable control group with nonneoplastic endocervical glandular epithelium. The ages of the patients with AIS ranged from 27 to 74 years (mean = 40). Mitotic figures were present in all AIS cases, and apoptotic bodies were seen in all but two extremely small lesions. In 28 AIS cases in which lesions were large enough to count 10 consecutive high-power fields (HPF), counts of apoptotic bodies ranged from 1 to 36/10 HPF (mean and median = 16), and counts of mitotic figures ranged from 1 to 53 mitotic figures/10 HPF (mean and median = 18). Counts of apoptotic bodies correlated directly with counts of mitotic figures. The ages of the 28 control patients ranged from 32 to 56 years (mean = 43). Counts of apoptotic bodies in the control cases ranged from 0 to 10 per case (mean = 1.1). Apoptotic bodies were present in only 13 (46%) control cases. The highest counts in these cases ranged from 1 to 6 apoptotic bodies/10 HPF (mean = 2.3). Mitotic figures were present in only 4 (14%) control cases. The counts in these cases ranged from 1 to 3 mitotic figures/10 HPF (mean = 1.8). Counts per 10 HPF were significantly (p < 0.001) more for AIS cases than for controls for apoptotic bodies and mitotic figures. Our results indicate that apoptotic bodies, as well as mitotic figures, occur almost universally in AIS. Both occur significantly more often and in greater numbers in AIS than in nonneoplastic endocervical glandular epithelium. Apoptotic bodies are a consistent morphologic feature of AIS, and their identification may be diagnostically useful.

Histopathologic findings in 107 uterine leiomyomas treated with leuprolide acetate compared with 126 controls.

The reported histopathologic findings in leiomyomas treated with leuprolide acetate (LA) differ. We examined 233 myomectomy specimens, including 107 myomas from 30 patients (mean age, 34.7 +/- 4.6 years) treated with LA. Their histopathologic findings were compared with those from a control group of 126 myomas from 30 untreated patients (mean age 32.7 +/- 5.3 years). The LA-treated leiomyomas had myxoid change (n = 2; 1.9%), total necrosis (n=4; 3.7%), focal necrosis (n = 5; 4.7%), calcifications (n = 5; 4.7%), hemorrhage (n = 8, 7.5%), vascular changes (n = 12; 11.2%), hydropic degeneration (n = 22; 20.5%), and hyalinization (n = 61; 57.0%). None of these changes differed significantly from the untreated controls. None of the LA-treated leiomyomas had nuclear atypia, whereas nuclear atypia occurred in four (3.2%) of the untreated leiomyomas; this difference was also not significant. Mitotic figures were present in 8.4% of the LA-treated myomas and 8.5% of untreated controls. The number of mitotic figures per 10 high-power fields was slightly higher in the untreated myomas, but the difference was not statistically significant (range, 0-3 for treated, 0-5 for controls). The degree of cellularity did not differ between the two groups. In conclusion, (a) LA-treated myomas do not significantly differ from untreated myomas with respect to nuclear atypia, calcification, total coagulative necrosis, focal coagulative necrosis, hemorrhage, vascular changes, myxoid change, hydropic degeneration, hyalinization, mitotic activity, or cellularity; and (b) the mechanism leading to a reduction in the size of myomas treated with LA is not apparent from routine histologic examination.

Presentation and management of bronchogenic cysts in the adult.

Bronchogenic cysts are congenital anomalies of the bronchial tree that are often asymptomatic at presentation in adults. Management of asymptomatic bronchogenic cyst in this population remains controversial. Eighteen patients with bronchogenic cysts were treated at our institution since 1975. At initial presentation, 10 patients (56 percent) were asymptomatic and 8 (44 percent) were symptomatic. Cough and pain were the most frequent symptoms. Two patients presented with potentially serious complications, one with respiratory distress from airway compression and the other with infection and airway fistulae. Chest radiographs were abnormal but nondiagnostic in 17 out of 18 (94 percent) patients. Chest computerized tomography (CT) scans were abnormal in eight of eight (100 percent) patients, but they confirmed the benign cystic nature in only five of eight (62.5 percent). Overall, considering the use of all imaging modalities and clinical suspicion, bronchogenic cyst was considered in the preoperative differential diagnosis in only 11 of 18 (61 percent) patients. Fifteen of 18 cysts were resected initially. Three of the asymptomatic patients who were followed up initially ultimately required resection because of the development of symptoms. A trend toward increased postoperative complications was noted in patients who were symptomatic at the time of surgery (27 percent vs 14 percent). In conclusion, adult patients with asymptomatic bronchogenic cyst may develop symptoms over time. Symptoms in adults can sometimes be potentially serious. Since a confident preoperative diagnosis is not always possible and because surgical complications may be more common in the symptomatic patient, we recommend surgical resection of all suspected bronchogenic cysts in operable candidates.

Pleural effusion resulting from metastatic papillary carcinoma of the thyroid.

We present a patient with a symptomatic pleural effusion resulting from papillary carcinoma of the thyroid metastatic to the pleura. Distinctive features include clinically evident pleural metastasis and the presence of psammoma bodies in the pleural fluid, which are characteristic (albeit not pathognomonic) of papillary carcinoma of the thyroid.

ThinPrep versus conventional smear cytologic preparations in the analysis of thyroid fine-needle aspiration specimens.

Paired fine-needle aspiration specimens were analyzed from 41 surgically resected thyroid nodules, to compare diagnostic accuracy, amount (absent, mild, moderate, or marked) and pattern (diffuse, droplets, or both) of colloid, nuclear detail (poor, satisfactory, or excellent) and cytoplasmic detail (intact or disrupted) in ThinPrep (TP) (Cytyc, Marlborough, MA) versus conventional smear (CS) cytologic preparations. The 41 surgical specimens included 25 colloid nodules, 6 papillary carcinomas, 4 follicular adenomas, 2 minimally invasive (encapsulated) follicular carcinomas, 3 Hashimoto's thyroiditis, and 1 Grave's disease. Both techniques identified seven of the eight carcinomas with the minimally invasive follicular carcinomas categorized as hypercellular follicular nodule, possibly malignant (HCFN). One papillary carcinoma was classified as a HCFN by both TP and CS techniques. The four follicular adenomas were classified as HCFN based on the TP slides. One oxyphilic follicular adenoma, associated with focal lymphocytic thyroiditis, was misinterpreted as Hashimoto's thyroiditis on a conventional smear. Three colloid nodules were interpreted as HCFN based on the TP slides. Two of these were similarly classified based on the conventional smear. ThinPrep slides contained less colloid and the colloid occurred as droplets rather than a diffuse pattern. TP slides had better nuclear detail but more often disrupted cytoplasm. In conclusion, the TP process does alter some cellular features; however, we experienced similar diagnostic accuracy with the TP and conventional smear preparations.

Recurrent osseous metaplasia of the cervix after loop electrosurgical excision.

BACKGROUND: Osseous metaplasia of the uterus is a rare pathologic entity usually affecting the endometrial cavity without cervical extension. It has been reported after abortion, in cases of chronic endometritis, and as a result of metabolic disorders as hypervitaminosis D, hyperparathyroidism, hypercalcemia, and hyperphosphatemia. CASE: A woman developed osseous metaplasia of the cervix shortly after loop electrosurgical excision procedure (LEEP) for severe cervical dysplasia. Normal transvaginal ultrasonograms before the LEEP, and failure to detect osseous metaplasia after histologic review of the tissue obtained from that procedure indicate that the osseous metaplasia had been induced by the LEEP. The heterotopic bone formation rapidly recurred after initial removal. Her serum calcium, phosphorus, and vitamin D levels were normal. CONCLUSION: LEEP may result in healing with metaplastic bone formation.

Cystic ovarian enlargement resulting from autoimmune oophoritis.

Autoimmune oophoritis is a rare cause of premature ovarian failure. Previous studies of autoimmune oophoritis have detailed the clinical, immunologic, and histologic findings. The gross pathologic features of the involved ovaries have detailed the clinical, immunologic, and histologic findings. The gross pathologic features of the involved ovaries have not been emphasized. Herein, we describe a 35-year-old woman with autoimmune oophoritis who presented with symptoms referable to a right cystic adnexal mass. She underwent a diagnostic laparotomy and right salpingo-oophorectomy for removal of an 8.0-cm-diameter, twisted multicystic ovary. Microscopically, mononuclear inflammatory cells infiltrated multiple follicular and luteal cysts. We have seen two additional patients with histologically verified autoimmune oophoritis who underwent diagnostic laparotomy and oophorectomy because of cystic enlargement of the ovaries. Stimulation of ovarian follicles by elevated levels of pituitary gonadotropins probably causes the cystic changes. Recognition of this aspect of autoimmune oophoritis may help to prevent oophorectomy in patients whose functioning ovarian tissue is already compromised by autoimmune destruction.

Delayed appearance of carbon granulomas of the scalp after use of carbon fiber pins for refixation of a stereotactic head frame. Case report.

The authors present a case involving the formation of several carbon granulomas in the scalp of a woman 7 years after she underwent craniotomy. Her operation had included the use of carbon fiber pins for refixation of a stereotactic head frame. Carbon granulomas have been noted in multiple organs following surgical or traumatic carbon deposition, but have not been reported in association with neurosurgical carbon fiber pins used for head fixation. The lesions in this case arose a few months after initiation of chemotherapy for the patient's brain tumor. The relationship of carbon and cutaneous granuloma formation to adjuvant therapies and treatment strategies is discussed.

Malignant mixed mullerian tumors of the ovary. An analysis of two long-term survivors.

Long-term survival following the diagnosis of ovarian malignant mixed mullerian tumor (MMMT) is unusual. This report analyzes two such long-term survivors. One patient presented with a FIGO Stage III, homologous MMMT treated initially with a combination of surgery and chemotherapy. Residual disease, present at the time of initial operation, responded to the chemotherapy; however, the tumor recurred 2 1/2 years postoperatively. This recurrence responded to a combination of surgery and chemotherapy, including continuous adjuvant chemotherapy. This patient is alive, on maintenance chemotherapy, and without evidence of disease, approximately 7 years after the recurrence and 9 years after the initial presentation. The other patient presented with a FIGO Stage III heterologous MMMT treated initially with combined surgery and chemotherapy. Residual disease was present at the time of initial operation. Persistent pelvic disease led to exploratory laparotomy, excision of an 8-cm diameter pelvic mass, and postoperative radiation therapy. The tumor recurred in the left supraclavicular lymph nodes 2 years later (3 years after the initial presentation). This recurrence responded to radiation therapy. This patient was last seen 2 years later (5 years after the initial presentation). At that time, she was without evidence of recurrence. She died 7 1/2 years after her initial presentation. These two patients represent examples of the unusual occurrence of patients with advanced-stage ovarian MMMT experiencing long-term survival following surgical and adjuvant therapy.

Juvenile granulosa cell tumors of the ovary.

The clinical and pathologic features of 13 cases of juvenile granulosa cell tumor were studied. Patients' ages ranged from 6 months to 56 years (median age, 17 years). Only one patient was postmenopausal. Three premenarchal patients had isosexual development. Five of seven postmenarchal patients had menstrual abnormalities, and two patients demonstrated virilization. Ascites was present in two patients. All patients had unilateral stage I tumors, ranging from 2.5 to 24.5 cm in greatest dimension (mean greatest dimension, 12.2 cm). Characteristic histologic features included nodular architecture, follicle formation, abundant interstitial and intrafollicular acid mucopolysaccharide-rich fluid, irregular microcysts, individual cell necrosis, and high mitotic activity (mean activity, 11 mitotic figures per ten high-power fields). The interstitial mucinous fluid consisted predominantly of hyaluronic acid. Immunohistochemical staining in five cases showed prominent positivity for vimentin (four cases), isolated cytokeratin AE1/3-positive cells (two cases), and nonreactivity for carcinoembryonic antigen and milk fat globule-2. Ultrastructurally, epithelial cells that resembled granulosa cells of the nonneoplastic preovulatory follicle and occasional cells with steroidogenic organelles were also found. Follow-up of ten patients revealed no tumor recurrences from six months to 33 years (mean, 9.5 years) after operation.

Endocervical adenocarcinoma in situ: an analysis of cellular features.

Cytologists increasingly encounter atypical endocervical cells, because of the increasing incidence of endocervical adenocarcinoma and the use of improved endocervical sampling devices. These atypical endocervical cells can cause diagnostic problems, especially in recognizing adenocarcinoma in situ (AIS) and distinguishing it from a variety of nonneoplastic changes. We analyzed 33 cervical smears from 22 patients with confirmed AIS and compared these to 19 cervical smears from 17 patients having atypical endocervical cells of undetermined significance and negative follow-up, including at least one tissue biopsy per case, to further investigate the cytologic features of AIS. The AIS smears typically had crowded three-dimensional cellular aggregates, with markedly hyperchromatic nuclei having altered polarity. Frequently, a minor component of AIS formed strips of distinctly columnar cells or sheets. Individual AIS cells occurred in 22 (67%) smears, but these were usually inconspicuous. The AIS smears also had increased nuclear to cytoplasmic ratios (100%), enlarged nuclei (94%), feathering (88%), rosettes (85%), nucleoli (76%), apoptosis (73%), mitoses (64%), multiple nucleoli (18%), and ciliated atypical cells (3%). Cytologic features occurring significantly (P < or = 0.001) more often in AIS cases were a predominance of three-dimensional crowded aggregates (79% vs. 32%), altered nuclear polarity in most groups (88% vs. 16%), marked hyperchromasia (91% vs. 16%), apoptosis (73% vs. 26%), an increased nuclear to cytoplasmic ratio (100% vs. 63%), feathering (88% vs. 26%), and individual atypical cells (67% vs. 16%). In summary, we identified a number of architectural and cellular features that occurred significantly more often in AIS cases than in cases having atypical endocervical cells of undetermined significance and negative follow-up.

ThinPrep vs. conventional smear cytologic preparations in analyzing fine-needle aspiration specimens from palpable breast masses.

Limited data exist concerning the cellular features of the ThinPrep (Cytyc Corp., Boxborough, MA) technique in the analysis of breast fine-needle aspiration specimens. Therefore, we analyzed a series of 75 surgically excised palpable breast masses and compared ThinPrep and conventional smear fine-needle aspiration preparations. Each mass was aspirated twice. The first sample was used for two alcohol-fixed conventional smears, and the second sample was rinsed into CytoLyt (Cytyc Corp., Boxborough, MA) solution for processing into a ThinPrep slide. The paired slides were separated and independently analyzed for adequacy, overall cellularity, single epithelial cells (absent, rare, moderate, or numerous), epithelial architecture (sheets or three-dimensional clusters), myoepithelial cells and stripped bipolar nuclei (present or absent), and nuclear detail (poor, satisfactory, or excellent). Each sample was classified as negative, negative consistent with fibroadenoma, atypical favoring benign, atypical favoring malignant, or positive for malignant cells. The 75 breast masses included 32 carcinomas and 43 benign lesions. Four conventional smears and one ThinPrep were unsatisfactory. Significantly, more conventional smears were limited by drying artifact (9 vs. 0). ThinPrep aspirates of carcinomas had better nuclear detail (P = 0.03) and greater cellularity (P = 0.05). ThinPrep aspirates of benign masses had greater epithelial cellularity (P = 0.007) and better nuclear detail (P < 0.001), and more specimens had myoepithelial cells (P = 0.007). The ThinPrep interpretation classified 29 of 32 carcinomas (91%) as positive and three as atypical favoring malignant (sensitivity = 100%). The conventional smear interpretation classified 28 of 31 carcinomas (90%) as positive and three as atypical favoring malignant (sensitivity = 100%). The ThinPrep interpretation classified 42 benign lesions as negative (23 cases), negative consistent with fibroadenoma (8 cases), atypical favoring benign (10 cases), and atypical favoring malignant (1 case) (specificity = 74%). The conventional smear interpretation classified 40 benign lesions as negative (25 cases), negative consistent with fibroadenoma (12 cases), and atypical favoring benign (3 cases) (specificity = 93%). ThinPrep was less specific, but the difference was not statistically significant (P = 0.065). In summary, ThinPrep aspirates had greater cellularity and better nuclear detail than conventional smears, and were just as sensitive in identifying the carcinomas. The difference in specificity between the two techniques was not statistically significant (P = 0.065). Diagn. Cytopathol. 1999;21:137-141.

Recurrent cervical squamous cell carcinoma presenting with cardiac tamponade. Recurrent cervical carcinoma-tamponade.

Cervical squamous cell carcinoma rarely metastasizes to the heart, and cardiac tamponade secondary to pericardial involvement has been only rarely reported. We describe a case of recurrent cervical squamous cells carcinoma presenting with cardiac tamponade secondary to extensive pericardial metastases. The patient, a 38-year old woman, initially presented with Stage IIIB cervical squamous cell carcinoma. She responded well to radiation and chemotherapy, there was no clinical or radiographic evidence of persistent disease after the initial therapy. Sixteen months after presentation, she developed shortness of breath and chest pain. The patient received additional chemotherapy; however, she died 17 months after her initial presentation. At autopsy, metastatic keratinizing squamous cell carcinoma extensively involved the pericardium and superficial myocardium. This case illustrates the unusual occurrence of recurrent cervical squamous carcinoma presenting with cardiac dysfunction secondary to pericardial metastases.

Osteosarcoma metastatic to the ovary: a case report and review of the literature.

A 23-year-old black woman presented with abdominal pain of sudden onset, high fever, chills, and an elevated serum alkaline phosphatase level. Examination revealed a tender abdominopelvic mass consistent with an ovarian mass. Her medical history was significant for an osteosarcoma of the left humerus removed 7 years earlier and excision of multiple pulmonary and chest wall metastases 2 years earlier. Exploratory laparotomy revealed a solid hemorrhagic left ovarian mass and ascites. There was no other evidence of disease. A left salpingo-oophorectomy was performed. Pathological examination of the mass showed metastatic osteosarcoma. Four months later, the patient died of widespread osteosarcoma. The clinicopathologic features of ovarian osteogenic sarcomas reported in the literature are reviewed. Pain, fever, and elevated serum alkaline phosphatase levels may be the presenting clinical features of this rare ovarian tumor.

Ovarian clear cell adenocarcinoma.

The clinical and pathologic features of 29 cases of ovarian clear cell adenocarcinoma (OCCA) were studied to evaluate outcome and potential predictors of survival. Patients' ages ranged from 30 to 89 years (median, 54 years). The study group, using the FIGO classification, consisted of 10 Stage I, 5 Stage II, 7 Stage III, 5 Stage IV, and 2 unstaged patients. Previous or concurrent endometriosis was noted in 45% of patients. Three patients (10.3%) had hypercalcemia. Only stage and the presence of macroscopic residual disease had a statistically significant association with survival. Stage I and II patients experienced a survival similar to a group of 305 patients with adenocarcinomas of non-clear cell type in our ovarian cancer registry with the exception of the very poor survival among Stage IC OCCA patients. Patients with Stage III and IV OCCA had a worse survival than non-OCCA registry patients of similar stage. Few objective responses to adjuvant chemotherapy could be demonstrated in these high-stage OCCA patients. Further study of postoperative adjuvant therapies is warranted.

Invasive adenocarcinoma of the cervix following LLETZ (large loop excision of the transformation zone) for adenocarcinoma in situ.

Adenocarcinoma in situ (AIS) of the cervix is a controversial entity which is being encountered with increasing frequency. Current critical issues in its management are the safety of uterine preservation in younger patients and the use of LLETZ conization. A 28-year-old patient was diagnosed with AIS and managed with LLETZ. Despite apparently negative margins of resection in the initial conizations, the patient was found to have invasive adenocarcinoma 1 year later. It is concluded, after review of the pathologic features of AIS, that if conservative management of AIS is elected, then standard cold knife conization should be performed and not LLETZ. Further study is required to establish the overall safety of conservative treatment of cervical AIS, including the use of LLETZ.

Undifferentiated nasopharyngeal carcinoma presenting as a parotid mass.

BACKGROUND: Nasopharyngeal carcinoma has a variety of presentations. However, in more than 50% of cases it may present with serous otitis or cervical metastases. Although the metastases usually occur in the neck, the lymphatic network of the parotid may also serve as a site for the metastatic deposits. We present a case in which the initial manifestation of nasopharyngeal carcinoma of the lymphoepithelioma type was a parotid mass. METHODS: Case study. RESULTS: A patient who was initially seen with an isolated parotid mass was found to have an undifferentiated carcinoma of the lymphoepithelial type. Further evaluation identified an occult nasopharyngeal carcinoma, and the patient underwent radiotherapy. CONCLUSIONS: A diagnosis of a malignant lymphoepithelial lesion of the parotid gland or undifferentiated carcinoma of the lymphoepithelial type mandates a search for an occult primary nasopharyngeal tumor.