Congenital generalized fibromatosis (renal and skeletal) with complete spontaneous regression

Teng, Paul; Warden, M. James; Cohn, Walter L.

doi:10.1016/s0022-3476(63)80045-1

Cited by 62 publications

(4 citation statements)

References 4 publications

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“…Contracture of these cells may be an important factor in the mechanism of spontaneous clinical regression previously described. 18,10,21, 25 Such contraction could also result in visceral pleural retraction, as noted in our case, and could produce a restrictive defect with progressive respiratory embar-rassment in cases with extensive pulmonary involvement.…”

Section: Discussionmentioning

confidence: 53%

Congenital generalized fibromatosis with visceral involvement. A case report

Roggli

Kim

Hawkins

1980

Cancer

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A case of congenital generalized fibromatosis in a black male infant with involvement of lungs, subcutaneous tissue, pancreas, adrenal, lymph nodes, and bone is described. Fifteen prior cases with pulmonary involvement are reviewed, emphasizing the poor prognosis associated with pulmonary lesions. This case demonstrated, in addition to previously described histologic features, broad bands of fibroblastic proliferation in the interlobular septa producing retraction of the visceral pleura. Possible etiologic factors are discussed, with suggestions for additional studies in future cases.Cancer 45:954-960, 1980. ONGENITAL GENERALIZED FIBROMATOSIS (CGF)C is a rare entity,23 involving the formation of nodules or masses of well differentiated fibrous tissue in the viscera, musculoskeletal system, and subcutaneous tissue. In fatal cases, death occurs in the first few months of life.1,15*19 In those infants who survive, spontaneous regression of the masses has been described.1s*20,21,2s Pulmonary involvement in CGF seems to carry a particularly grave prognosis. Fifteen cases of fibromatosis involving the lungs have been ~e S c~~~e~, 2 ,~,~, R , 1 0 ' " 4 . ' " . ' " . 2 2 -2 4 , 2~, Z 7 and all but 2 patients died.x*19 We report a case of CGF with pulmonary involvement in a black infant. Case ReportA 4-month-old black boy was seen because of tachypnea and a one-week history of fever and anorexia. The infant, who weighed 1500 g at birth, was born to a primigravida woman after 35 weeks of gestation. Two purplish-red masses on the posterior aspect of the boy's head and the left side of the neck noted at birth were thought to be hemangiomas.Physical examination revealed that the infant was afebrile with a respiratory rate of 100 and pulse of 140imin. His weight, 4275 g, was below the 3rd percentile. The occipital mass had completely regressed, and the left cervical mass measured 5 x 5 x 1 cm. Right lower lobe rales, a Grade IIiVI systolic ejection murmur, and an S, gallop were noted on auscultation. Hepato-splenomegaly was present. Electrocardiographic studies demonstrated right axis deviation and right ventricular hypertrophy. The chest x-ray showed a n interstitial infiltrative process. The hemoglobin was 10.6 gidl, hematocrit 3296, white blood count 69001pL1, with 41% segmented neutrophils, 40% lymphocytes, 5% band neutrophils, and 14% monocytes. An open lung biopsy from the right upper lobe showed minimal interstitial mononuclear inflammation, mild intraalveolar edema, and Type I1 pneumonocyte hyperplasia. On the seventh postoperative day, the infant suffered a cardiac arrest and could not be resuscitated. Autopsy FindingsThe striking findings were in the respiratory and cardiovascular systems. The surface of the lungs was grossly nodular; the lungs were firm and noncrepitant. Some of the interlobular septa were prominently thickened by gray-white tissue, and small white spherical nodules, 1 mm or less in diameter, were occasionally noted in the parenchyma of the lung (Fig. 1). The heart was enlarged, with right ventricu...

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Section: Discussionmentioning

confidence: 53%

Congenital generalized fibromatosis with visceral involvement. A case report

Roggli

Kim

Hawkins

1980

Cancer

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“…Multiple infantile myofibromatosis with visceral involvement or generalized (multicentric lesions arising not only on the skin and muscle but also on the bone, lung, heart, and most frequently gastrointestinal tract) 3. Spontaneous regression of tumors (Teng et al 1963;Schaffzin et al 1972;Brill et al 1982), possibly due to apoptotic cell death (Fukasawa et al 1994). (Inwards et al 1991) 1.…”

Section: Cd34-positive Infantile Myofibromatosismentioning

confidence: 99%

Infantile Myofibromatosis

Chen¹

2016

Atlas of Genetic Diagnosis and Counseling

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“…Although apparent visceral lesions indicate a poorer prognosis., this separation into two groups seems artificial and not particularly useful otherwise. Some cases with proved visceral involvement go on to complete re-gression (Teng et al 1963), and it is probable that non-symptomatic visceral lesions occur in other patients who have complete regression.…”

Section: Natural Historymentioning

confidence: 99%

Congenital generalized fibromatosis: an autosomal recessive condition?

Baird

Worth

1976

Clinical Genetics

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Congenital generalized fibromatosis is a rare condition which is often misdiagnosed and given an erroneously poor prognosis. Five new cases are presented in this report, all initially having been diagnosed as neurofibromatosis. The histopathological findings are presented and the differential diagnosis is discussed. The natural history of the disorder appears to include an initial phase of proliferation soon after birth with appearance of new tumor masses. Providing these do not involve vital viscera, the patient survives with regression and eventual disappearance of all lesions. Spontaneous regression occurred in all five patients reported. Two sets of sibs occurred in the cases described. The possible genetic aspects of this are discussed and the pertinent literature reviewed.

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Congenital generalized fibromatosis (renal and skeletal) with complete spontaneous regression

Cited by 62 publications

References 4 publications

Congenital generalized fibromatosis with visceral involvement. A case report

Congenital generalized fibromatosis with visceral involvement. A case report

Infantile Myofibromatosis

Congenital generalized fibromatosis: an autosomal recessive condition?

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