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D C Dahlin

Publications and source records attributed to D C Dahlin.

At least 55 records · Page 3Linked to original sources

Bizarre parosteal osteochondromatous proliferations of the hands and feet.

We report 35 cases of peripheral skeletal osteochondromatous tumefactions which are both histologically and radiologically distinctive. The ages of the patients ranged from 14 to 74 years, with no sex predilection. Symptoms were related to the tumefaction. None of the patients had antecedent physical trauma or radiation. All lesions occurred on proximal phalanges, metatarsals, or metacarpals. Primary treatment was usually excision, and the gross appearance was typically that of a small osteochondroma. Histologically, the lesion exhibited marked proliferative activity, irregular bony-cartilaginous interfaces, and enlarged, bizarre, and binucleate chondrocytes, mimicking chondrosarcoma. Radiologically, the proliferations lacked both central continuity of the tumor with the underlying osseous medulla and "flaring" of the adjacent cortices. In 18 cases, the lesions recurred after primary excision, and at least eight lesions recurred twice. In spite of the rate of recurrence and the disturbing histologic appearance of these proliferations, behavior as a malignant tumor is either very rare or nonexistent.

Adolescent↗

Giant-cell tumor of the sphenoid bone. Review of 10 cases.

Giant-cell tumor rarely affects the sphenoid bone. Ten cases of this tumor seen at the Mayo Clinic are reviewed, bringing the number of cases in the world literature to 31. These tumors, which present in the second and third decades of life and are slightly more frequent in women than in men, may present with various symptoms, including headache, visual field defects, blindness, and diplopia. The symptom complex and roentgenographic findings are seldom, if ever, sufficiently characteristic to allow preoperative diagnosis. The microscopic differential diagnosis of giant-cell tumor of the sphenoid region includes giant-cell reparative granuloma, aneurysmal bone cyst, fibrous dysplasia, and "brown tumor" of hyperparathyroidism. Interesting features of this series include the presence of multifocal giant-cell tumor in one case, and the absence of Paget's disease of bone in every case. Complete follow-up review in these cases allowed a discussion of therapy and prognosis; it appears that subtotal excision with postoperative radiation therapy is the treatment of choice. While the histological appearance of giant-cell tumor is characteristic, caution must be exercised in interpreting small samples, and various reparative and metabolic disorders must be considered in the differential diagnosis.

Adolescent↗

Malignant bone tumors associated with neurofibromatosis.

Neurofibromatosis (von Recklinghausen's disease), a phakomatosis inherited as an autosomal-dominant trait, predisposes to multiorgan neoplasia as well as to skeletal abnormalities. In an attempt to define more clearly the nature of osseous malignant lesions associated with von Recklinghausen's disease, we reviewed three cases of coexistent neurofibromatosis and malignant tumors of bone. None of the tumors was considered neurogenic. One patient had a fibrosarcoma, and two patients had malignant fibrous histiocytomas. No gross relationship to nerves was demonstrated, and special studies for axons were negative. Primary neurogenic sarcomas of bone are extremely rare; the diagnosis should be considered only when the tumor originates from an intraosseous nerve or contiguous neurofibroma. The occurrence of primary bone sarcomas in patients with neurofibromatosis seems to be a sporadic event.

Adult↗

Osteosarcoma of extragnathic craniofacial bones.

Osteosarcomas of extragnathic craniofacial bones--those bones of the skull excepting the jaw bones--are rare lesions, constituting fewer than 2% of all osteosarcomas. In our series of 21 patients (12 male and 9 female), the ages ranged from 6 to 77 years, and 10 patients were in the third or fourth decade of life. At least six patients had predisposing conditions: Paget's disease of bone in three and prior regional irradiation in three. Most of these tumors were high-grade lesions, and most were extensive when treated. There were 11 osteoblastic, 6 fibroblastic, and 1 small cell variant; 2 lesions had features resembling malignant fibrous histiocytoma. One tumor, originally interpreted as grade 4 osteosarcoma, was unavailable for variant classification. Surgical removal, irradiation, and chemotherapy were used in treatment, alone or in combination. Only two patients survived 5 years, and one of these patients died of her disease after 65 months. Almost half of our patients died within 1 year after diagnosis. The one long-term survivor (12 years) underwent a radical surgical procedure when her tumor was small; this was the only patient in this series who had a low-grade lesion. Because of the characteristics described, osteosarcoma of extragnathic craniofacial bones must be considered a distinct disease.

Adolescent↗

Chondrosarcoma arising in osteochondroma.

Secondary chondrosarcomas are rare. An analysis of 75 cases of chondrosarcomas secondary to osteochondroma (42 lesions were from the Mayo Clinic files and 33 were from consultation files) revealed that 40 of the patients had single exostosis and 35 had multiple lesions. More males than females were affected, and most of the patients were age 20 to 40 years. The tumors involved various bones. Malignant change was manifested radiologically by fuzzy margins of the cartilage cap and by the presence of lucent zones within the lesion. Grossly, the surface of the thickened cartilage cap is irregular. Microscopically, most of the tumors were well-differentiated (Grade 1). Treatment was surgical. Simple excision of the tumor resulted in a large recurrence rate. Resection and amputation were generally curative. Only 12 of the patients have died of their tumor. Most patients died of local recurrence. Only two of the 75 patients had definite evidence of metastatic disease.

Adolescent↗

Aneurysmal bone cyst and other nonneoplastic conditions.

Aneurysmal bone cyst is a benign proliferative tumefaction of bone. Histologic similarities indicate a kinship among classic aneurysmal bone cysts, essentially "solid" proliferative lesions in bones; giant cell reparative granulomas of the jaws, at the base of the skull, and in the small bones of the hands and feet; skeletal lesions of hyperparathyroidism; and even pseudosarcomatous myositis ossificans, proliferative myositis, and proliferative fasciitis.

Adolescent↗

Hemangiopericytoma of bone.

Hemangiopericytoma primary in bone is a rare tumor. A review of 15 cases (five from the Mayo Clinic files and 10 from the consultation files) revealed that all tumors were unifocal and that the most common location was the pelvis (three lesions in the sacrum and two in the ischium). The series involved seven male and eight female patients whose ages ranged from 14 to 74 years. Histologically, all of the tumors were composed of cells with round to oval nuclei arranged around vascular spaces. On the basis of cellularity, nuclear atypia, and mitotic activity, four tumors were benign, two intermediate, and nine malignant. Surgery was the primary treatment for the 15 patients. Of the 14 patients with adequate follow-up, eight died of disease, three were alive with recurrent disease, and three were alive and disease-free.

Adolescent↗

Hemangioendothelial sarcoma of bone.

Hemangioendothelial sarcoma of bone is a rare tumor. A series of 112 cases was studied to define the clinical and pathologic features of this disease. Of the various histologic features evaluated to define the ones most significant prognostically, the histologic grade of the lesions was the single feature that correlated best with disease-free survival. In this series, patients with multi-focal tumors did not have a better prognosis than patients with unicentric disease. Surgery remains the primary treatment for these patients. Radiotherapy may be useful for surgically inaccessible lesions of the axial skeleton. Too few patients have received adjuvant chemotherapy to evaluate its usefulness in this setting.

Adolescent↗

Langerhans' cell granulomatosis (histiocytosis X) of bone in adults.

Langerhans' cell granulomatosis (LCG) (histiocytosis X) of bone is generally considered a childhood disease. A series of 61 adult patients who presented with histologically confirmed skeletal disease was studied. The male-to-female ratio was 2.1, and 36 patients were more than 30 years old, the oldest being 71 years. Pain was the most frequent symptom. Forty-six patients has solitary lesions, six had two or three, and nine had more than three. The time interval between the development of lesions, although usually less than 2 years, ranged up to 32 years. Rib, skull, and pelvis were the most frequently involved sites. Eight patients had extraskeletal disease. Radiologically, a well-defined lytic lesion, either with no surrounding reaction or with sclerotic margins, was seen most often. The rare, poorly defined lesions tended to occur in the clavicle. Microscopically, typical Langerhans' cells were present in large numbers. Eosinophils were usually prominent. Benign giant cells, mitotic figures, and necrosis also were observed. There was no histologic difference between solitary and multiple lesions. Surgery, radiation therapy, or a combination of both usually provided adequate treatment. Only two of the solitary lesions recurred. Eleven patients suffered from chronic, progressive disease, which inconsistently responded to treatment. None of the patients in our series died of the disease.

Adolescent↗

Melanotic neuroectodermal tumor of infancy: a malignant tumor of the femur.

An 18-month-old white girl had a malignant melanotic neuroectodermal tumor of infancy of the left femur. Light microscopic findings of a biopsy specimen of the femoral lesion demonstrated the characteristic histologic features, that is, nests of small cells with hyperchromatic nuclei and scant cytoplasm lying within cleftlike spaces lined by cuboidal, melanin-producing cells. Electron microscopy confirmed both neuroblastic and melanocytic differentiation. Despite radiation and chemotherapy, an extensive pelvic tumor developed, which prompted laparotomy 20 months after the femoral biopsy, The apparently metastatic tumor was largely necrotic, but viable nests of undifferentiated small cells and rare individual melanin-containing cells were identified. The patient died 4 months later, at 3 years of age. This case is of particular importance because it is the first example of this tumor found in a long bone. No similar lesions were seen in 17,000 primary tumors of bone treated and seen in consultation at this institution. Its aggressively malignant clinical course is also unusual; only 6 of the 159 cases of melanotic neuroectodermal tumor of infancy reported to date have expressed malignant behavior.

Female↗

Does Peutz-Jeghers syndrome predispose to gastrointestinal malignancy? A later look.

Several reports have recently suggested an association between the development of intestinal carcinoma and the presence of Peutz-Jeghers syndrome. During the 45-year period between 1935 and 1979, 48 patients with Peutz-Jeghers syndrome were seen at the Mayo Clinic, Rochester, Minn, and followed up for a median period of 33 years. Carcinoma of the small or large intestine did not develop in any patient, with possibly one exception. Survival of patients with Peutz-Jeghers syndrome also was found to be similar to that of the general population. Therefore, skepticism continues about the premalignant potential of this rare syndrome, especially in the small intestine, and surgical conservatism should be practiced in its management.

Adult↗

Case report 160.

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Brain Neoplasms↗

Postradiation sarcoma of bone: review of 78 Mayo Clinic cases.

Postradiation sarcoma of bone is an uncommon but serious sequela of radiation therapy. Seventy-eight Mayo Clinic patients have been treated for sarcomas arising in irradiated bones. They received their initial radiotherapy for a wide variety of nonneoplastic and neoplastic conditions, both benign and malignant. Thirty-five sarcomas arose in bone that was normal at the time of radiotherapy, and 43 arose in irradiated preexisting osseous lesions. The latent period between radiotherapy and diagnosis of sarcoma averaged 14.3 years. Ninety percent of the postradiation sarcomas were either osteosarcomas or fibrosarcomas; chondrosarcoma, malignant (fibrous) histiocytoma, malignant lymphoma, Ewing's tumor, and metastasizing chondroblastoma also occurred. Prompt radical surgery, when feasible, is usually the treatment of choice for the sarcoma. About 30% of patients with sarcomas of the extremities or craniofacial bones survived 5 years without recurrence; there were no disease-free survivors among patients with tumors of the vertebral column, pelvis, or shoulder girdle. The low risk of sarcoma following radiotherapy for the treatment of cancer should not be a contraindication to its use in these patients; however, radiation therapy for benign bone tumors should be reserved for lesions that are not amenable to surgical treatment. An unusual case is also reported herein in which a fibrosarcoma was discovered in the humerus of a patient who had received radiotherapy 55 years previously for a verified osteosarcoma in the same site.

Adolescent↗

Management of osteogenic sarcoma: a perspective based on the Mayo Clinic experience.

We reviewed pertinent aspects of the Mayo Clinic experience with osteogenic sarcoma to place into perspective some of the recent advances in the management of patients with this tumor. Various histopathologic subtypes have been recognized and can be differentiated from the conventional or classic osteogenic sarcoma. Our experience indicated that, when appropriate, crossbone amputation provided adequate and effective surgical treatment. A controlled study in which pulmonary micrometastases were evaluated failed to demonstrate that prophylactic pulmonary irradiation after amputation conferred any therapeutic advantage. The use of combination chemotherapy or transfer factor did not improve disease-free survival when the results were compared with our recent experience with surgical treatment alone. Progressive improvement in disease-free and total survival was observed between 1963 and 1974, although we have been unable to relate this to the effects of adjuvant chemotherapy. Aggressive surgical excision of pulmonary metastatic lesions had a positive effect on total survival. These observations led us to conclude that carefully controlled studies are still needed to define more clearly the role of adjuvant chemotherapy in nonmetastatic osteogenic sarcoma. Although such a study is in progress at the Mayo Clinic, it is too early for any evaluation of the results.

Antineoplastic Agents↗

Pathological fractures through non-ossifying fibromas. Review of the Mayo Clinic experience.

Twenty-three cases of a pathological fracture through a lesion verified histologically as non-ossifying fibroma were seen over a forty-nine-year period. The average age of the patients at the time of fracture was twelve years. All fractures except one were located in the lower extremity, most frequently in the distal end of the tibia (ten). The percentage of bone occupied by the fibroma in the transverse plane exceeded 50 per cent on both the anteroposterior and the lateral radiographs in every patient. The vertical length was always the maximum dimension and in all non-fibular lesions exceeded thirty-three millimeters. Treatment consisted of cast immobilization with biopsy at a later date, simple curettage, curettage and autogenous bone-grafting, or segmental resection of fibular lesions.

Adolescent↗