Congenital peribronchial myofibroblastic tumor: case report of an asymptomatic infant with a rapidly enlarging pulmonary mass and review of the literature.

BACKGROUND: Congenital peribronchial myofibroblastic tumor (CPMT) is a rare, benign lung tumor of infants, with only 19 reported cases worldwide. It is often diagnosed by prenatal imaging or in the immediate postnatal period due to co-morbidities like polyhydramnios, fetal hydrops, respiratory distress, and heart failure. OBSERVATION: We report the oldest known infant (8 weeks old) diagnosed with CPMT, and present his clinical course including the relevant radiographic and histopathologic findings. CONCLUSIONS: CPMT is a rare tumor that should be considered among other primary lung tumors of infancy (developmental, benign, and malignant) even if not detected prenatally or in the immediate postnatal period.

Congenital granular-cell myoblastoma.

The clinical and pathologic features of congenital granular-cell myoblastoma in five infant girls are reported. One lesion, treated expectantly, progressively decreased in size and after 3 yr and 9 mo could not be detected, while two lesions which were imcompletely excised did not recur. It is suggested that congenital granular-cell myoblastoma is caused by an intrauterine stimulus, and that this stimulus may possible be production of estrogen by the fetus. Congential granular-cell myoblastoma should be treated expectantly or by limited excision, and has an excellent prognosis.

[Congenital granular-cell tumor]. / Vrozhdennaia zernistokletochnaia opukhol'.

Tumour consisting of nodes (15 x 10 x 8 mm, 2 cm and 0.3 cm in size) on the alveolar processes of the maxilla and mandibula is found in a newborn. Histological diagnosis is granular-cell myoblastoma.

[Single congenital granular cell myoblastoma (congenital epulis) of the gum]. / Mioblastoma de células granulosas congénito solitario (épulis congénito) de la encía

A case of granular-cells (congenital epulis type) in the gum in a female newborn is reported. The differential characteristics between this tumor and the "so-called granular cell myoblastoma" are described.

Congenital peribronchial myofibroblastic tumor: a case report.

We describe a rare occurrence of congenital peribronchial myofibroblastic tumor of the lung presenting in early fetal life. A female patient in the 24th week of gestation who presented with polyhydramnios was admitted for examination. Ultrasound examination revealed a mass compromising the lungs. Because the intrauterine fetal death was revealed by the ultrasound, delivery was induced. Necropsy revealed a pulmonary lesion compromising the left lower pulmonary lobe together with hepatomegaly. Microscopic analysis of the lung showed a lesion with a storiform arrangement of spindle cells with focal peribronchial distribution. Hepatic architecture was diffusely altered by fibrotic tissue. Immunohistochemical analysis on the pulmonary lesion showed high vimentin positivity in the fusocellular components, pointing to the mesenchymal nature of the lesion. Significant differentiation of smooth muscle tissue, as indicated by high HHF35 positivity, was also observed. Electron microscopy on the pulmonary lesion revealed elongated cells with some cytoplasmatic processes, a finding that is also compatible with mesenchymal differentiation.

Fetal hydrops associated with congenital pulmonary myofibroblastic tumor.

Abstract We report on a fetus with a congenital pulmonary myofibroblastic tumor, the prenatal detection of which with imaging modalities has not been reported up until now. A 32-year-old woman was referred to our hospital at 29 weeks' gestation because of severe fetal hydrops. Sonograms and magnetic resonance imaging showed a large solid tumor in the left thorax. The fetus died in utero the next day. Autopsy confirmed that the tumor was confined to the lower lobe of the left lung, and circulatory insufficiency from compression by the tumor was considered to be the cause of fetal hydrops and demise. Histologic examination revealed that the tumor was composed of uniform short spindle cells with no atypia and a large number of vessels. In addition, with immunohistochemical studies, the tumor cells were stained for calponin but not for cluster differentiation (CD)-31, CD-34, alpha-smooth muscle actin or S-100.

Congenital granular-cell neoplasms. An unusual case report with ultrastructural findings and a review of the literature.

Congenital granular-cell tumors are uncommon benign neoplasms of unknown cause that develop primarily in gingival tissue and usually do not recur after surgical excision. Untreated, these neoplasms either cease to grow or "spontaneously" regress after birth. In this report, we describe an unusual case of a child with several congenital granular-cell neoplasms that arose on the lips and continued to increase in size as the child grew. Histologic examination of the neoplasms revealed them to be composed of granular and mesenchymal cells associated with abundant collagen fibers and prominent vascular structures. Ultrastructural study disclosed typical granular cells and a preponderance of immature mesenchymal cells, some of which appeared to be transitional or early granular cells. We interpreted these findings to mean that primitive mesenchymal cells are the proliferative elements and precursors of granular cells in congenital granular-cell neoplasms. The presence of numerous immature mesenchymal cells corroborates the clinical impression that the lesions were growing. Based upon the histological and ultrastructural findings reported here, and a review of the literature, we favor a mesenchymal or endothelial-cell origin for congenital granular cell neoplasms.

[Neumann's tumor or congenital epulis of the newborn]. / Tumeur de Neumann ou épulis congénitale du nouveau-né.

Congenital epulis is a rare benign gingival tumor affecting mainly female neonates. Histology shows characteristic granular cells. Although diagnosis and therapy fail to raise particular problems, this is not the case for histopathogenesis of lesion. Two cases observed recently in West Africa are reported.

Congenital smooth muscle hamartoma of the skin.

A 2-month-old white girl had a congenital, tan-colored, slightly elevated, 3 x 2-cm plaque on the left midback. Clinically, the lesion was suspected to be a solitary mastocytoma. Rubbing the lesion produced a transient erythema and edema that was similar to Darier sign seen in mastocytoma. Analysis of a skin biopsy specimen revealed a smooth muscle hamartoma; special stains did not show evidence of increased numbers of mast cells. This relatively uncommon condition can be confused with a variety of other cutaneous diseases. Light microscopic examination of a skin biopsy specimen establishes the diagnosis. A review of the approximately 50 cases reported in the literature showed that there is no known associated systemic involvement or malignant transformation. The clinical lesions usually become less prominent with time.

[Congenital epulis of the newborn (granulous Abrikosov tumor). Remarks apropos of a personal case]. / Epulis congénitale du nouveau-né (tumeur granuleuse d'Abrikossoff). Commentaires à propos d'une observation personnelle.

The authors report a case of middle and superior gingival tumor in a newborn patient. Macroscopic and topographic aspects are a "congenital epulis". The surgical treatment is easy. Histologically finding shows a benign tumor with granulous cells called "Abrikossoff Tumor". The literature is reviewed to study histological aspect and pathogeny of this tumor. They try to distinguish the macroscopic aspect of the "congenital epulis" with the histologically finding. It is not systematically a granulous cells tumor.

[Benign gingival granular cell tumor in the newborn: congenital epulis]. / Tumor gingival benigno de células granulares en el recién nacido: epulis congénito.

Gingival granular cell tumor, or congenital epulis, was first described by Neumman in 1871 and subsequently 201 cases have been published in 173 patients. It is an uncommon benign tumor that is present like a pedunculated, smooth surfaced, isolated lesion on the alveolar mucosa of the maxillar of the newborn child. Its firm consistency and variable size can occasionally cause problems in the child's breathing or feeding. This tumor is easily diagnosed clinically and, although spontaneous regression of the tumor mass has occasionally been reported, the current treatment is surgical removal. Two new cases of congenital epulis are reported and a literature review is included.

Congenital gingival granular cell tumor with smooth muscle cytodifferentiation.

Congenital epulis of the newborn is a rare benign congenital gingival granular cell tumor (GGCT) of unknown histogenesis which occurs most commonly on the gingiva of the anterior maxillary alveolar ridge in girls. The granular cells in this entity are histologically indistinguishable from those in extragingival granular cell tumors, known historically as granular cell myoblastoma (GCM), which occur at any age and appear to be of Schwann cell origin. Ultrastructural, histochemical, and immunohistochemical features of three GGCT were examined and compared to three GCM and a granular cell ameloblastoma. This is the first instance in which the ultrastructure of granular cells in a congenital epulis showed evidence of smooth muscle differentiation. Carcinoembryonic antigen-like immunoreactivity was localized in granular cells from all granular cell tumors studied, but S-100 protein was present only in GCM. The smooth muscle ultrastructural features and the lack of S-100 protein in GGCT strongly suggest a different histogenesis from that of GCM. The GGCT is likely derived from a primitive gingival perivascular mesenchymal cell with the potential for smooth muscle cytodifferentiation.