Localized esophageal leiomyoma and hypertrophic osteoarthropathy

Localized

Esophageal Leiomyoma Osteoarthropathy

By R. Massicot,

D. Aubert,

A. Mboyo, Besanqon,

l The authors report the case study of a 13-year-old girl, suffering from epigastric pain related to an important infectious syndrome and from marked hypertrophic pneumosteopathy (clubbed fingers, diffuse cortical hyperostosis). Radiological examination (chest x-ray and computed tomography scan) showed a partly calcified right posterior mediastinal mass. The esophagoscopy showed a fistular emanation from the esophagus to the tumor. The biopsy results were of no diagnostic significance, and the samples demonstrated a bacterial and fungal colonization. An endoscopic sonogram showed an inhomogeneous mass with hyperechoic structures developed between the mucosa and the outer muscular coat of the esophagus. The patient underwent surgery, which showed a large intraparietal tumor of the esophagus, which, through pathological examination, was confirmed to be a benign leiomyoma. Postoperative follow-up was simple and the pneumosteopathic symptoms regressed rapidly. This disease is rare in children under 15 years of age. It is only exceptionally associated with a hypertrophic pneumosteopathy. This pathology is diagnostically challenging to the surgeon. The endoscopic ultrasonography is the best procedure in the assessment of subepithelial tumor and extrinsic compressions of the oesophagus. Copyright o 7997 by W.B. Saunders Company

INDEX WORDS: Esophageal mosteopathy, endoscopic

leiomyoma, sonogram.

hypertrophic

pneu-

L

EIOMYOMA of the esophagus is rarely seen in children, localized fotis are by far the rarest encountered. We report on a localized leiomyoma associated with a hypertrophic osteoarthropathy in a 13-yearold girl. To our knowledge this exceptional association has not been previously reported in children.

and Hypertrophic

0. Destuynder, France

and P.E. Queneau

and feet was seen in radiographs (Fig 2). A chest x-ray showed a right postero-inferior mediastinal clacified mass. Computed tomography (CT) scan indicated a central (5 X 5 cm) cavity with a large fistula in the esophagus (Fig 3). This cavity, situated on the right side of the last third of the esophagus was largely opacified during the barium meal. During esophagoscopy the fistula was seen, the biopsy specimens showed only bacterial and fungal colonization of an unspecified inflammatory tissue. An endoscopic sonogram showed a inhomogeneous mass with hyperechoic structures developed between the mucosa and the outer muscular coat of the esophagus (Fig 4). A right thoracotomy through the sixth interspace was performed. A m-m, circumscribed and yellowish mass, adherent to the esophagus, was found and resected. The fistula was sutured. The chest was drained. The recovery was uneventful. Histological examination showed a benign leiomyoma (5 X 5 cm) with a central necrotic cavity. Pain and edema regressed dramatically a few days after surgery. At follow-up examination 6 months later the girl was doing well, and there were no more signs of hypertrophic osteopathy. The contrast study showed a normal esophageal passage of barium. In childhood, leiomyomas account for nearly 3% of all tumors. Only 2.6% of esophageal leiomyomas are diagnosed during childhood.’ Leiomyoma is the most common esophageal benign tumor in children. Contrary to the sex distribution and the predominant localized forms in adults, leiomyomas are 1.7 times more common in girls, and the localized forms are rarer (9%) in children.’ The majority of the leiomyomas occur in the middle and lower third of the esophagus. The association between leiomyoma and hypertrophic osteoarthropathy is exceptional; Ulla12 reported on a 49-year-old man presenting a hypertrophic osteoarthropathy and a (9- X 5- X 4-cm) leiomyoma of the lower half of the esophagus. The relief from joint symptoms was complete 2 days after surgery, and 1 year later there remained only a slight clubbing of fingers and toes. We have observed the same course but faster; 1 month after removal of the tumor the signs of hypertrophic osteopathy had totally regressed.

CASE REPORT A 13-year-old girl was referred to our institution for epigastric pain enhanced by respiratory movements and for painful and swollen forearms, wrists, and ankles. The child presented with frequent sudden rises of temperature and a 1.5.kg weight loss in 15 days without dysphagia or cough. Physical examination showed an inflammatory clubbing of fingers (Fig l), and a light trembling of the extremities. Subperiostal new bone formation at the forearms, metacarpals, legs,

From the Service de Chin&e Infantile, Pr Aubert, Hbpital St Jacques, and the Service de Gastro-Ente’rologie, Pr Curayon, Hapita Jean Minjoz, 25030 BesanGon, France. Address reprint requests to Pr D. Aubert, Service de Chirurgie Pkdiatrique (Pr Aubert), H6pital St Jacques, F 25000 Besangon, France. Copyright o 1997 by WB. Saunders Company Fig 1.

0022-3468/97/3204-0031$03.00/O

646

Journal

of Pediatric

Clubbing

Surgery,

of the fingers.

Vol32,

No 4 (bpril),

1997:

pp 646-647

ESOPHAGEAL

LEIOMVOMA

AND

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OSTEOARTHROPATHV

Fig 4. Endoscopic ultrasonographic photograph delineated inhomogeneous lesion beyond the mucosa. The outer muscular coat is uninterrupted

Fig 2. new bone

Radiograph formation.

of the

fibula

and

the

tibia

shows

subperiostal

The diagnosis of a right postero-inferior mediastinal calcified mass with a central cavity fistulized in the esophagus was difftcult to establish. Chest x-ray, upper gastrointestinal study, CT scan, and esophagoscopy could not distinguish between a fistulized adenopathy, a

shows a sharply normal esophageal over the tumor.

leiomyoma and an infection of a bronchogenic cyst, an esophageal duplication, or a pulmonary sequestration. Biopsies were performed but were not helpful. They are considered hazardous and not recommended by some investigators.’ the lesion may be difftcult to reach with a standard biopsy forceps3 Imaging of the esophageal wall is only possible with endoscopic ultrasonography (EUS), which is therefore the best procedure in the assessment of subepithelial tumor and extrinsic compressions of the esophagus. The echo patterns of leiomyomas have been already well described: local thickening of the muscularis propria or presence of a mass in the muscular coats with sharp boundaries and without involvement of the mucosa. Small masses are hypoechoic whereas lesions greater than 2 cm may present some inhomogeneity and hyperechogeneity. The outer muscular coat can often be seen uninterrupted over the tumor.3,b Because of its clear imaging of the intramural abnormalities. we advocate endoscopic ultrasonography as the better procedure in the diagnosis of esophagus tumors or compressions.

REFERENCES

Fig 3. Computed nal calcified mass

with

tomography a central

scan cavity

shows fistulized

right posterior mediatito the esophagus.

1. Bourque MD: Spiland N, Bensousan AL, et al: Esophageal leiomyoma in children: Two case reports and review of the literature. J Pediatr Surg 24:1103-1107, 1989 2. Ullal SR: Hypertrophic osteoarthropathy and Jeiomyoma of the esophagus. Am J Surg 123:356-358, 1972 3. Tio TL, Tytgat GNJ, den Hartog Jager FCA: Endoscopic ultrasonography for the evaluation of smooth muscle tumors in the upper gastrointestinal tract: An experience with 42 cases. Gastrointest Endosc 36:342-350, 1990 4. Amouyal P, Amouyal G, Palazzo L; et al: L’echoendoscopie: Une nouvelle technique d’exploration des tumeurs soustpitheliales et des compressions extrinseques oeso-gastriques. Gastroenterol Clin Biol 14:8-14, 1990