The spleen in the spleen syndrome: the association of splenoma with hematopoietic and neoplastic disease--compendium of cases since 1864.

Splenomas have been described as benign congenital growths. We report six cases of splenomas, all in males. Three had hairy cell leukemia, one had Hodgkin's disease, and one had myeloid metaplasia. Average splenic weight was 1629 gm, and all had multiple splenomas (average diameter 1.89 cm). Splenomas bulged above the cut splenic surface, were pseudoencapsulated, and showed involvement of the underlying disease. A re-evaluation of 105 prior cases are adult (86%; age: 49), with 56% associated with neoplastic disease or hematologic abnormalities. Average spleen weight was 626 gm, and 22% contained multiple splenomas. We conclude that splenomas are most likely an acquired proliferative process strongly associated with neoplastic disease or hematologic abnormalities. Splenomas can also be congenital growths (hamartoma; 26% of the series), which are of little importance.

Primary hyperparathyroidism associated with two enlarged parathyroid glands.

An increasingly recognized although small percentage of patients with primary hyperparathyroidism have enlargement of two parathyroid glands. We have treated nine patients with primary hyperparathyroidism associated with such double parathyroid gland enlargement. In four of these patients, marked asymmetry of the two enlarged glands was noted and the failure to recognize and excise a second enlarged parathyroid gland resulted in persistent or recurrent hyperparathyroidism. In one of these patients, the second enlargement was present in a super-numerary mediastinal gland. The subsequent excision of the second enlarged parathyroid gland resulted in normocalcemia in each instance. This contrasts with five patients in whom initial excision of two enlarged glands resulted in normocalcemia with no recurrence of hypercalcemia. Only three patients fulfilled the histologic criteria of true double adenomas. The remainder showed multiglandular hypercellularity. This experience supports identifying all parathyroid glands and recognizing that even minimal enlargement of a gland may be important pathophysiologically, regardless of its histopathologic classification. Excision of both enlarged glands, even if asymmetric, is appropriate.

Diagnosis of adrenal ganglioneuroma in pregnancy with magnetic resonance imaging and ultrasonography. A case report.

A right adrenal gland mass was found during sonographic examination of the right upper quadrant during a workup for hyperemesis gravidarum. Magnetic resonance imaging was used to clarify the origin of the mass. The tumor was nonfunctional. Because of the possibility of a malignancy, the patient underwent a successful second-trimester exploratory laparatomy with right adrenalectomy. Pathology demonstrated a benign ganglioneuroma.

Epithelioid hemangioendothelioma. A rare tumor with variable prognosis presenting as a pleural effusion.

The chest x-ray film a 22-year-old man showed a large right-sided pleural effusion that was grossly hemorrhagic when aspirated. A computerized tomographic scan showed a complex mass with cystic components contiguous with the diaphragm. On thoracotomy the mass was found to be arising from the diaphragm and had the consistency of an organizing hematoma. Pathologic studies showed the mass to be an epithelioid hemangioendothelioma. This rare tumor has never been reported previously as arising from the diaphragm. It has a variable prognosis, but surgery remains the treatment of choice. In this report, we review the clinical and pathologic characteristics of this unusual tumor, as well as the distinctive roentgenographic findings with which it presented.

Surgical treatment of dermatofibrosarcoma protuberans.

The clinical course and histopathologic factors of 50 consecutive patients treated for dermatofibrosarcoma protuberans were reviewed. Forty-eight patients were observed until the present time or death. No patient had distant metastases develop, although 16 patients had 18 recurrences of the dermatofibrosarcoma protuberans at the site of initial therapy. There was no correlation between the diameter of the primary lesion and the incidence of recurrence. There was no correlation between the histologic pattern of invasion and recurrence. However, a trend toward decreasing recurrence was noted with increasing minimal margins of resections (41 per cent less than 2 centimeters versus 24 per cent greater than or equal to 2 centimeters). The lowest incidence of recurrence (20 per cent) was noted with minimal margins of resection greater than or equal to 3 centimeters. Five year recurrence free survival rates increased with increasing margins of resection--59 per cent less than 1 centimeter; 66 per cent greater than or equal to 1 centimeter; 70 per cent greater than or equal to 2 centimeters, and 80 per cent greater than or equal to 3 centimeters. No patient had distant metastases and no change in histologic pattern was noted with progressive local recurrence.

Primate mammary development. Effects of hypophysectomy, prolactin inhibition, and growth hormone administration.

The pituitary gland has been found to be an important factor in mammary development in primates. Hypophysectomy in 12 sexually immature monkeys caused significant inhibition of estradiol (E2)-induced mammary growth and development. A histological index of mammary development in sexually immature hypophysectomized animals was lower (0.82) than in intact E2-treated controls (3.4; P less than 0.008). Hypophysectomy also inhibited growth of the mammary gland as judged by a size index. Despite the hypophysectomy, E2 stimulated some, albeit blunted, mammary growth and development, which may have been due to incomplete hypophysectomy. Selective inhibition of prolactin by ergot drugs in intact animals did not prevent full mammary development, suggesting that there may be pituitary mammogens other than prolactin, or that very low or unmeasurable concentrations of prolactin were sufficient to synergize with E2 to cause full acinar development. The mean histological index was 3.08 in E2-treated animals and 3.16 in animals treated with E2 plus pergolide. There was also no difference in the size of the glands. We evaluated the effect of growth hormone on mammary development by treating three hypophysectomized animals with pure 22,000 mol wt human growth hormone (hGH) (Genentech, Inc., South San Francisco, CA). We found that physiological or slightly supraphysiological concentrations of hGH in animals with unmeasurable prolactin were incapable of restoring the capacity of E2 to induce full mammary growth. These findings suggest that, if growth hormone is a mammary mitogen, that physiological concentrations are insufficient to synergize with E2 to induce full mammary growth or that other forms of hGH are mammogenic. Our studies suggest that the role of the pituitary gland in mammary mitogenesis in primates is more complicated than previously thought. They also raise the possibility that heretofore unidentified pituitary substances may be mammogenic.

Small intestinal lymphoma in three patients with acquired immune deficiency syndrome.

Three cases of small bowel lymphoma in young homosexual men are presented. All three had acquired immune deficiency syndrome as demonstrated by demography, sexual history, cachexia, opportunistic infections by Cytomegalovirus, Pneumocystis carinii, atypical Mycobacterium, Candida, and/or evidence of immune deficiency, such as skin test anergy, lymphopenia, inversion of T-helper/T-suppressor ratio, and diminished lymphocyte response to either phytohemmaglutinin or pokeweed mitogen. All had peripheral and/or abdominal lymphadenopathy, and gastrointestinal symptoms, e.g., diarrhea, spasms, constipation, and oral candidiasis. The diagnosis of lymphoma was made at laparotomy in all cases. All three had complete removal of localized tumor (stage Ie or IIe), yet died within 6 months of surgery and/or chemotherapy. Thus gastrointestinal complaints may not always be related to "gay bowel" syndrome, or other infectious diseases in patients with acquired immune deficiency syndrome. Small intestinal lymphoma should be added to the list of neoplasms to which this group is susceptible.

Adjuvant postoperative radiation therapy for Dukes C adenocarcinoma of the rectum.

This study was designed to explore the effects of adjuvant postoperative radiation therapy on the course and survival of patients with Dukes C adenocarcinoma of the rectum. Moderate dose radiotherapy was offered to 64 patients. Twenty-four accepted and were treated, while the remainder refused. With a mean follow-up of 32.3 months, the overall survival rate was 63% (40/64) and the mean disease-free survival rate at the time of this report was 45%. The following variables were analyzed separately: operative procedure, distance of the tumor from the anal verge, tumor size, and transmural, vascular, lymphatic and perineural invasion of tumor. The most significant differences between irradiated and non-irradiated patients were found in the group of patients whose lesions were 6 cm or less from the anal verge. Of the 19 such patients with an average follow-up of 36.4 months, ten patients were irradiated and nine were not irradiated. The irradiated group had a 90% (9/10) survival rate and 70% (7/10) of them were disease free; the non-irradiated group had a 44% (4/9) survival rate and 22% (2/9) of them disease free. It is concluded that patients with adenocarcinoma of the rectum metastatic to lymph nodes, whose lesions' lower border is measured 6 cm or less from the anal verge, benefit significantly from adjuvant postoperative radiotherapy.

Transmural fluid movement in the freshly excised human gallbladder.

Direct information concerning the incidence and nature of gallbladder dysfunction in symptomatic cholethiasis was obtained by examining the rate and direction of transmural fluid movement in the freshly excised gallbladder. Each specimen was emptied of bile, cannulated and filled with a measured volume of bicarbonated Ringer's solution, suspended in the same solution at 37 degrees C., gassed with 95 per cent oxygen and 5 per cent carbon dioxide and weighed at ten minute intervals for at least one hour. Of the 42 human gallbladders examined, concentrating activity, ranging from 1 to 20 per cent of intraluminal volume per hour, was found in 14 and no activity in 17. Direction of fluid transport was found to be completely reversed in 12 additional gallbladders, four of which presented as hydrops. Histologic alterations ranged from mild to severe and could not be correlated with functional status. Application of indomethacin to the solution bathing both surfaces of 13 nonfunctioning, or partially functioning, gallbladders restored or increased function in eight. It can be concluded from this study that the concentrating activity is retained in approximately 30 per cent of patients with symptomatic cholelithiasis; cholesterol stones form in gallbladders with normal concentrating activity; the spectrum of gallbladder dysfunction ranges from simple loss of concentrating activity to hydrops, the latter reflecting complete loss of this activity plus secretion into the lumen, and dysfunction is probably induced by chemical mediators of inflammation rather than by structural changes.

Primary melanoma thickness correlated with regional lymph node metastases.

We studied 119 patients with stage I primary cutaneous malignant melanoma, who were undergoing regional lymph node dissection, to determine the relationship of lymph node metastases to thickness of the primary lesion. The lymph nodes in the dissection specimen were each evaluated by serial sections. None of the patients with lesions less than 1.0 mm thick had nodal micrometastases. When lesions exceeded 1.0 mm in thickness, there was no appreciable increase in the incidence of nodal metastases until a thickness greater than 4.0 mm was reached, in which cases the incidence of metastases was 50%. Predictive variables were determined by multiple logistic regression analysis. Only lesions that were at least 4.0 mm thick and were not located on the upper extremities were significant predictors of lymph node metastases; within this category there was a 64% incidence of lymph node metastases.

Churg-Strauss syndrome. Development of cardiomyopathy during corticosteroid treatment.

A patient with Churg-Strauss syndrome had pulmonary lesions, vasculitis, gastrointestinal tract involvement, and leukocytosis with hypereosinophilia. A wedge section from the lung showed necrotizing granulomata filled with necrotic eosinophils and fibrinoid, and rimmed by giant cells and eosinophils. Arteries and veins of all sizes were affected with perivascular eosinophilic-rich, palisaded, giant cell granulomata with evidence of partial or complete occlusion. The patient was treated with prednisone, with excellent resolution of his symptoms. Recurrent episodes of pneumonia and wheezing responded to increased levels of prednisone plus bronchodilators and antibiotics. Cardiomyopathy developed after two years while taking prednisone. His condition has been managed with digoxin, diuretics, and increased amount of prednisone. Although our patient has shown clinical improvement in his cardiac status, objective studies show minimal resolution.

A method for the dissection of lymph node specimens (anatomic method).

A method called the "anatomic method" of dissecting lymph nodes in a variety of cancer specimens, is presented. It is found to be comparable to the clearing method in terms of detecting lymph nodes and is unencumbered by the slow turnaround time and economic inconvenience. The anatomic method is described in four steps, illustrated by a schematization of the technique. Its advantages over the clearing method include: 1) it is time saving; 2) it is conducted on the fresh state specimen; 3) it is economical; 4) it reduces the monotony often associated with such dissections; 5) it serves as a teaching exercise. Adoption of the method by pathologists is recommended.

Heterotopic salivary tissue and branchial cleft sinus.

Bilateral lesions which appeared clinically to be branchial cleft sinuses were removed from a patient's neck. One was indeed a branchial cleft sinus, with elements of salivary tissue, but the other proved to be pure salivary tissue with a duct characteristic of a salivary gland. The embryology and clinical characteristics of heterotopic salivary glands are discussed. The occurrence of a heterotopic gland together with a branchial cleft sinus supports the contention that the heterotopic glands arise through errors of development in the branchial apparatus.

Desmoplastic malignant melanoma: A light and electron microscopic study of two cases.

This light and electron microscopic study of two recent cases of desmoplastic malignant melanoma (DMM) attempts to resolve the conflict in views regarding the nature of the cells responsible for the desmoplasia associated with this clinicopathologic entity. On the basis of evidence presented, it is concluded that the cells are dedifferentiated tumor cells with fibroblastic features and probably functions, rather than host engendered fibroblasts in response to invasive melanoma. The evidence includes: observation of macular desmosomes between tumor cells, an unheralded feature previously noted in amelanotic and melanotic melanomas; electron microscopic observation of fibroblast-like cells by others in spindle cell squamous carcinomas; and light microscopic features of malignancy including vascular invasion in one of the two cases. A reproducible light microscopic pattern diagnosis of this variant of malignant melanoma is reaffirmed in both cases.

Desmoplastic malignant melanoma: study of a case by light and electron microscopy.

This article is a study by light and electron microscopy of a case of desmoplastic malignant melanoma that attempts to resolve the conflict in views regarding the nature of the cells responsible for the desmoplasia associated with this clinico-pathologic entity. On the basis of evidence presented, it is concluded that the cells are dedifferentiated cells of the malignant melanoma with fibroblastic features and probably fibroblastic functions rather than host-engendered fibrolbasts in response to invasive melanoma. The evidence consists of the finding of macular desmosomes between those cells, a feature previously noted in amelanotic and melanotic melanomas, the finding of others by electron microscopy of fibroblast-like cells in spindle-cell squamous carcinomas, and the finding by light microscopy of features of vascular invasion of the malignancy. That the diagnosis of this variant of malignant melanoma by pattern can be made by light microscopy is reaffirmed.

Angiographic patterns in renal oncocytomas.

Renal oncocytomas are benign tumors arising from proximal tubular epithelial cells. They appear radiographically as solid masses which are vascular on angiography. Angiograms of 13 cases of renal oncocytomas were reviewed, as well as those of 155 renal-cell carcinomas. The classic angiographic findings for the oncocytoma include a spoke-wheel pattern, a homogeneous nephrogram, and a sharp, smooth rim. The finding of a homogenous blush and/or a spoke-wheel pattern greatly increases the possibility of an oncocytoma, though a renal-cell carcinoma may have any or all of the classical findings described for an oncocytoma.

Desmoplastic malignant melanoma: a report on two additional cases.

Two cases of desmoplastic malignant melanoma are described, bringing the total reported to nine. The first developed in a lentigo maligna melanoma and contains the seed of its local recurrence in the primary lesion. The second developed in a clinically apparent congenital nevus and unites the histologic features of the desmoplastic variant with the epithelioid and spindle cell histology of its primary, side by side in a local recurrence. Both primaries were clinically recognized pigmented lesions while the desmoplastic recurrences were amelanotic. Other clinico-pathologic characteristics of DMM as previously described, are confirmed. Light microscopic evidence is presented supporting the view that the desmoplastic reaction is a rare manifestation elicited by a highly invasive spindle cell component of malignant melanoma.

Proximal tubular adenomas of kidney with so-called oncocytic features. A clinicopathologic study of 13 cases of a rarely reported neoplasm.

Four renal "oncocytomas" were observed between July 1974 and January 1975 at University Hospital. A review of all renal cell neoplasms previously classified as carcinomas since 1952 was conducted to determine whether this cluster of tumors represented a recent increase in incidence or whether other renal "oncocytomas" had gone unrecognized in the past. The 23-year review of 194 carcinomas yielded an additional nine "oncocytomas." None had appeared prior to 1964, whereas almost half were found in the 1973-1975 interval alone. While it appears that some "oncocytomas" of the kidney have gone unrecognized in the past, it is also evident that their recent increased incidence is epidemiologically significant. Renal "oncocytomas" and renal cell carcinomas are compared with respect to morphology, clinical presentation, and biologic behavior. Evidence derived from this comparison permits the conclusion that these neoplasms originate from proximal tubular epithelium, are benign clinicopathologic entities, and henceforth should be called proximal tubular adenomas with so-called "oncocytic" features.