The patient was a 58-year-old male who had smoked (40 cigarettes/day) and drunk (250g/ day) heavily for more than 30 years. He came to our hospital on the 10th April 2019 for treatment because of repeated upper abdominal fullness and discomfort. He had suffered from pulmonary tuberculosis 30 years ago and the symptoms disappeared after treatment. On the 20th October 2017, doppler ultrasound examination suggested multiple hepatic masses; MHHs were considered. The patient had no symptoms of chest discomfort. His mental state was good, and there had been no significant loss of weight. There was no history of chest trauma. His granddaughter had a congenital hemangioma on her forehead.
Gastroscopy revealed esophagitis, erosive gastritis and duodenitis. Abdominal doppler ultrasound revealed multiple hepatic masses again; these were considered to be MHHs again. Plain chest CT scan images showed two adjacent masses on the left chest wall, with slightly uneven density and sizes of 1.9 x 1.3cm and 1.1 x 0.9cm respectively. CT values ranged from − 10HU to 10HU, with an average of 1HU. The masses protruded into the thorax and were surrounded by a "capsule". These were laterally connected to the chest wall with a broad base and reached the 5th intercostal region. The normal intercostal muscle structure was interrupted and had disappeared. These were thought to be chest wall cysts or localized encapsulated effusions(Fig. 1A). There was an old pulmonary tuberculosis in the upper lobe of the right lung. In addition, scattered small nodules in both lungs and bilateral pleural thickening were considered as inflammatory changes. The patient also had bronchitis and emphysema. Abdominal plain CT revealed multiple slightly low-density nodules and masses in the liver with clear boundaries; the largest of these was about 3.5cm x 2.8cm.
According to the chest contrast-enhanced CT (CECE) scan images, in the arterial phase (Fig. 1B,1D), the two adjacent masses presented with nodular/tubular enhancement (CT values ranged from 0HU to 34HU, with an average of 16HU), and in the delayed phase (Fig. 1C,1E), the contrast agent presented further enhancement of filling (CT values ranged from 0HU to 40HU, with an average of 29HU). The intercostal artery ran along the lower margin of the "capsule" and the lateral edge of the two masses. The CECT diagnosis suggested that these may be neurogenic tumors or hamartomas, even though they exhibited the typical "fast in, slow out" and progressive filling enhancement. Hemangiomas are rare in our region. According to the abdominal CECT scan images, the edges of the hepatic masses were significantly enhanced in the arterial phase (Fig. 2G). The portal vein phase (Fig. 2H) and the delayed phase (Fig. 2I) gradually filled and strengthened to the center. This was typical of the enhancement associated with hepatic hemangioma; thus, these were considered as MHHs. Physical examination revealed slightly coarse breath sounds in both lungs. Laboratory examinations showed no evidence of thrombocytopenia or abnormal coagulation.
The surgeon performed VATS exploration on the patient, and found a “tumor” of approximately 3 x 2 cm in size with a clear boundary and a complete capsule. The intercostal nerve traversed the capsule, and two tumors were visible inside when the capsule was opened; the tumors were then completely removed. In addition, multiple pleura adhesions were found in the left thoracic cavity and were severed. The intraoperative diagnosis was “left posterior mediastinal tumors/neurogenic tumors?”. Pathological findings suggested hemangiomas (Fig. 3J,3K). We invited two chief physicians from the Department of Pathology to classify the tumors independently, and for both masses, they gave the diagnosis of venous hemangioma (VH). However, the origin of the tumors had not been confirmed by pathological examination; the surgically removed tissues had been discarded and could not be examined.
We strongly suspected that the clinician had made a mistake in his description of the location of the tumors; these are not associated with the posterior mediastinum according to relevant literature [1]. The surgeon told us that the surgical record was written by a doctor who was undergoing standardized training. At this time, it had been too long since the surgery, and the surgeon could not accurately describe the location of the tumors. Pathologists had referred to the location of the tumors described by the clinicians and did not make an independent judgment. Therefore, two chief radiologists in our team (both of these have been engaged in imaging diagnosis for more than 20 years) judged the location of the tumors from the CT images independently. The radiologists strongly agreed that the tumors originated from the chest wall intercostal space. Normally, the intercostal artery passes behind the corresponding intercostal space between the intrathoracic fascia and the intercostal intima. The intercostal nerve runs underneath the corresponding intercostal artery. A review of CT scan images showed that the intercostal artery ran along the lower edge of the “capsule” and the lateral edge of the two masses; the normal intercostal muscle structure was interrupted and disappeared. According to the surgical records, the "capsule" was intact, and the intercostal nerve traversed the "capsule" (hemangiomas are known not to have capsules). Therefore, the "capsule" contained parietal pleura and intercostal intima (at least) and these two VHs represented tumors of the intercostal area. According to the literature, these should have originated in the intercostal muscles.
The patient presented with multiple hepatic masses without any symptoms of hepatic discomfort. The patient's two doppler ultrasound findings within two years and the CT scan results prior to VATS were considered to reveal MHHs. Therefore, surgical resection of the hepatic masses was not performed; 32 months later, CT showed no significant changes in the hepatic masses.
Literature review
We searched the English literature relating to IH from January 1990 to January 2022 and found 17 case reports [2–18]; the present study represents the 18th case report (Table 1). By screening these case repor ts we identified the involvement of 10 males and 8 females. Seven patients were under 30 years old, one patient [7] was found to have hemangioma in infancy (2-year-old); nine patients were affected on both the left and right side. Three patients had VH, five patients had cavernous hemangioma (CA), two patients had arteriovenous hemangioma (AVH); three patients had mixed hemangioma (MH); there was one case of intramuscular hemangioma of small-vessel type (SVIH) and one case of intramuscular hemangioma of large-vessel type (LVIH). The subtypes were not specified in three cases. Hemangioma was successfully diagnosed by magnetic resonance imaging (MRI) in four cases and by CT in two cases. Phlebolith was detected in two hemangiomas [5, 8]. Eleven patients were without chest symptoms; 2 patients were not specified. Two patients had a history of trauma, two patients had other vascular lesions and five patients had rib destruction.
Table 1
Characteristics of patients in published English literature
Author | Age, sex | Size (cm) | Intercostal space | Symptoms of chest | imaging diagnosis | Rib destruction | Pathology | Follow up | medical |
CT | MRI | history |
Wincheste [2] | 39y, F | 5 | Right (5) | Discomfort, fullness | Unclear | - | N | CA | - | - |
Robinson [3] | 58y M | 3 | Right (3) | No symptoms | Pleural-based discrete mass | - | N | CA | - | - |
Hashimoto [4] | 32y, M | 1.2 | Left (6) | No symptoms | Neurogenic tumour | Hemangioma | N | VH | N (24m) | - |
Dzian [5] | 36y, M | 9.5X9x3 | Left (7 ~ 8) | Pain | Unclear | Hemangioma | Unclear | VH | N (10m) | - |
Dantis [6] | 18y, M | 6x4.5x4 | Left (7) | Swelling, pain | Unclear | - | Y | CA | N (12m) | - |
Ulku [7] | 11y, F | 8.5x7.5x5.5 | Right (9 ~ 11) | Unclear | Unclear | Unclear | N | CA | N (6m) | - |
Saldanha [8] | 34y, F | 3x2 | Left (3) | Unclear | Hemangioma | - | Unclear | CA | N (12m) | - |
Elbawab [9] | 14y, M | 6x4x3.5 | Right (5) | No symptoms | Unclear | Unclear | Y | Hemangioma | N (6m) | - |
Agarwal [10] | 44y, F | unclear | Right (3) | No symptoms | Unclear | Hemangioma | N | Hemangioma | N (18m) | - |
Mei [11] | 14y, F | 7x6x5 | Right (4) | Pain, hemoptysis | Pulmonary metastasis | - | Y | Hemangioma | N (10m) | Trauma |
Aguilo [12] | 23y, F | 4x5 | Left (7) | No symptoms | - | - | N | AVH | - | Subdural AVM |
Kara [13] | 46y, M | 4 | Right (2) | No symptoms | Hemangioma | - | N | AVH | N (48m) | - |
Yuan [14] | 44y, F | unclear | Left ( 2) | No symptoms | Vasculopathy | Unclear | N | CA + CH | N (36m) | - |
Ono [15] | 33y, M | 9x9.5 | Right (3 ~ 5) | No symptoms | - | Unclear | N | SVIH + LVIH | N (36m) | - |
Ali [16] | 22y, F | 7x7x6 | Left (6 ~ 9) | No symptoms | Unclear | Unclear | Unclear | CA + CH | N (6m) | - |
Yonehara [17] | 33y, M | 5x5 | Left (6) | No symptoms | Tumors of bone/cartilage | - | Y | SVIH | N (60m) | Trauma |
Kubo [18] | 27y, M | 5.5x3.5 | Right (7) | Pain, exertional dyspnea | Unclear | Hemangioma | N | LVIH | N (6m) | - |
Current case | 58y, M | 3x2 | Left (5) | No symptoms | Neurogenic tumor/ Hamartoma | - | Y (after 32m) | VH | Y (32m) | MHHs |
y, years; m, months; M, male; F, female; Y, yes; N, no; AVM, arteriovenous malformation. |