Laryngeal kaposiform hemangioendothelioma: Case report and literature review

Auris Nasus Larynx 37 (2010) 258–262 www.elsevier.com/locate/anl

Laryngeal kaposiform hemangioendothelioma: Case report and literature review Dong Wook Kim a, Jin Haeng Chung b, Soon Hyun Ahn c, Tack-Kyun Kwon a,* a

Department of Otorhinolaryngology, Seoul National University College of Medicine, Seoul National University Hospital, Republic of Korea b Department of Pathology, Seoul National University Bundang Hospital, Seongnam, Republic of Korea c Department of Otorhinolaryngology, Seoul National University Bundang Hospital, Seongnam, Republic of Korea Received 5 March 2009; accepted 8 May 2009 Available online 23 June 2009

Abstract The kaposiform hemangioendothelioma (KHE) is an uncommon vascular neoplasm of borderline or intermediate malignant potential in which involvement of the head and neck region is rare. A 5-year-old girl was admitted to the emergency room with abrupt massive hemoptysis. Transoral laryngoscopy revealed a round, reddish mass with active bleeding on the anterior portion of the left vocal fold region. Since re-hemoptysis and large amount of aspiration caused breathing difficulties for the patient, suspension exam was performed under general anesthesia. Mass excision and thorough bleeding control was performed with laser assisted laryngo-micro system. Postoperatively, there was no further bleeding. The pathologic diagnosis of the tumor was KHE. To the best of our knowledge, this is the first report of a KHE in the larynx presenting as recurrent hemoptysis in childhood. # 2009 Elsevier Ireland Ltd. All rights reserved. Keywords: Kaposiform; Hemangioendothelioma; Larynx; Hemoptysis

1. Introduction Hemoptypsis is a rare presenting symptom in children. Detectable hemoptypsis may be substantial because children tend to swallow their bloody sputum. The diagnosis of pediatric hemoptysis can be challenging and urgent. Generally the etiology of pediatric hemoptypsis differs from that in adults. Although hemoptysis in adults is often caused by bronchiectasis, bronchogenic carcinoma, or tuberculosis, pediatric hemoptysis is more associated with infection, tracheostomy problems, or aspirated foreign body. Most cases are mild and self-limited. However, the potentially life-threatening pulmonary vascular anomalies and congenital heart disease should be identified. Rarely, tumorous condition in airway should also be taken into account in the differential diagnosis. In diagnosis and management of hemoptypsis, the otolaryngologist is in a * Corresponding author. Tel.: +82 2 2072 2448; fax: +82 2 745 2387. E-mail address: [email protected] (T.-K. Kwon).

unique position. We report a rare case of a 5-year-old girl with hemoptypsis due to a kaposiform hemangioendothelioma (KHE) is an uncommon vascular neoplasm of borderline or intermediate malignant potential.

2. Case report On April 26th, 2005, a 5-year-old girl was admitted to hospital emergency room with abrupt massive hemoptysis and respiratory discomfort. At the time of admission, the massive hemoptysis was spontaneously controlled to some degree. However, a small amount of fresh throat bleeding persisted and began to overflow. Although the patient did not complain of dyspnea after the cessation of massive hemoptypsis, she had to continually clear her throat and spit out saliva mixed with fresh blood in order to relieve respiratory discomfort and muffled voice. The patient was found to have a mild inspiratory stridor but either subcostal, jugular retractions or wheezing were not present. She had

0385-8146/$ – see front matter # 2009 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.anl.2009.05.003

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Fig. 1. On laryngeal endoscopic exam, a 1 cm sized red-colored bleeding mass was detected in the left supraglottic area (A). An enhanced mass lesion in left supraglottis could be seen the axial view of the neck CT (B). (For interpretation of the references to color in this figure legend, the reader is referred to the web version of the article.)

experienced symptoms of hoarseness and intermittent hemoptysis which had begun about 2 weeks earlier. After checking her vital sign, routine evaluation including complete blood count profile, coagulation tests and simple chest film were performed and a prolonged aPTT (75.5 s) and leukocytosis (WBC 21,610/ml and neutrophil 74%) were noted. Oral intake and drinking were prohibited, close care was taken to maintain intravenous access and adequate fluid replacement. After she was stabilized, a

laryngoscopic examination was performed by an ENT doctor. On laryngeal endoscopic exam, a 1 cm sized redcolored, bleeding mass was detected in the left supraglottic area. In order to perform an accurate exam, repetitive examinations were tried with the patient clearing her throat multiple times. There was no other abnormality in larynx. Computed tomography disclosed a solitary well enhancing mass lesion in left supraglottic area and patent airway (Fig. 1).

Fig. 2. Under suspension exam, the bleeding mass (arrow) was located in the left ventricle. Metal suction tip (arrow head) (A). Hemostasis and removal of mass was achieved with CO2 Laser (B). Immediate post operative finding (C). The removed hemangioma was dark red and 0.7 cm  0.5 cm in size (D). (For interpretation of the references to color in this figure legend, the reader is referred to the web version of the article.)

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Fig. 3. The tumor contained nests of cells with small slit-like spaces and intracellular lumina. and solid nests of round to slightly spindle shaped endothelial cells (H&E stain, 100). Immunoreactivity for CD31 was present in most of tumor cells (immunohistochemistry stain, 400).

The patient was brought to the operating room for bleeding control. Under general anesthesia, orotracheal intubation was successfully done although mass bleeding continued during the intubation. Under suspension microlaryngoscopy, a reddish vascular tumor with irregular surface was detected. It had originated from the left laryngeal ventricle. With meticulous bleeding control, the mass was excised using CO2 laser assisted laryngo-micro system (Fig. 2). Postoperatively, there was no further bleeding or dyspnea. The patient’s voice was also preserved. The pathologic diagnosis of the tumor was benign hemangioendothelioma. The hematoxylin–eosin stain showed an endothelial cell neoplasm with spindle area and intracellular lumen formation. Additional immunohistochemistry study also revealed strong immunoreactivity for endothelial markers such as CD31, vimentin and focal positivity for CD34. These findings were consistent with hemangioendothelioma, favoring the kaposiform type (Fig. 3). For 3 years after the operation, there has been no signs of recurrence.

3. Discussion The term ‘‘Kaposiform hemangioendothelioma (KHE)’’ was introduced by Zukerberg et al. in 1993 [1,2]. Although other vascular pediatric tumors such as cavernous hemangioma, juvenile hemangioma, and angiomatosis, are benign, KHE shows intermediate or borderline malignant features and may be placed in between benign hemangiomas and highly malignant angiosarcomas. Local aggressiveness and failure to spontaneously regress are clinical features of KHE. Distant metstasis is rare [1,2]. Although the diagnosis of KHE is often made only on histopathological and immunohistochemical grounds, biopsy is rarely performed in KHE due to the risk of hemorrhage [3]. Therefore, most diagnoses are made through surgical excision of the tumor. These clinical features make decisions regarding treatment difficult.

KHE has histopathological characteristics common to benign capillary hemangiomas and Kaposi’s sarcomas. Some lesions possess vessels with round or oval lumens with strong similarity to benign capillary hemangiomas, whereas other lesions, even within the same tumor, are similar to Kaposi’s sarcomas and are composed of spindled neoplastic endothelial cells with attenuated nuclei that form elongated or crescentic vascular lumens. KHE also produces intracellular and extracellular hyaline globules similar to those of Kaposi’s sarcoma. Also, both KHE and Kaposi’s sarcomas express CD34 [2,4]. KHE may be differentiated from Kaposi’s sarcoma and benign capillary hemangioma in that there is a lack of both periodic-acid-Schiff-positive globules and well-formed spindle cell fascicles [5]. KHE typically occurs in infancy and childhood [6]. Of the biopsy-proven or probable cases of KHE, the most commonly involved sites were cutaneous lesions of the limbs and trunk, occurring in 75% of cases, followed by retroperitoneal involvement, occurring in 18% [7]. Also, very rare cases occurred in the head and neck area such as the face, oropharynx, ethmoid sinus and internal auditory canal [1]. However, a larynx or hypopharynx origin of KHE is extremely rare, and to our knowledge, our case is the first report of KHE involving the larynx in English literature. KHE is frequently associated with consumption coagulopathy (Kasabach–Merritt syndrome), characterized by profound thrombocytopenia, life-threatening hemorrhage, and lymphangiomatosis [4]. The site and extent of the disease greatly influences the biologic behavior of KHE and associated coagulopathy [2]. Although infant retroperitoneal KHE associated Kasabach–Merritt syndromes are mostly complicated, peripherally occurring KHE can still be associated with Kasabach–Merritt syndrome. Lyons et al. reported that 44% of cases with KHE did not show the Kasabach–Merritt syndrome, as was the case in our patient [4]. Radiologically, hemangioendothelioma in the head and neck regions has been reported to have an aggressive destructive tendency, particularly at the temporomastoid region [5,6]. KHE may contain vascular structures indicating

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Table 1 Kaposiform hemangioendothelioma of the otorhinolaryngologic area (19 patients). Patient [reference]

Age

Sex

KMS

Location

Size

Surgery or radion therapy

Medication

Outcome

Follow-up

1a 2 [9] 3 [1]

5 yr 19 yr 6 mo

F M M

No No No

1 cm  1 cm 1 cm  1 cm N/A

ANED ANED ARD

3 yr 1 yr N/A

8 yr

F

No

No

ANED

1.5 yr

5 [11]

3 yr

M

No

No

ANED

N/A

6 7 8 9

36 mo 6 mo 1 mo 21 mo

M M M M

No No Yes No

1 cm  1 cm N/A N/A N/A

Excision (CO2 laser) Excision Embolization, partial resection Embolization, excision (endoscopy) Hemithyroidectomy, neck dissection Excision No No Excision

No No Steroid, IFN

4 [10]

No IFN Steroid, IFN No

ANED DOD ARD ANED

1 1 5 4

[4] [4] [4] [4] [4] [13] [3] [5]

4 yr 4 yr 5 mo 4 mo 1 yr 1 mo 1 mo 12 mo

M M F M F M F F

N/A No Yes Yes Yes Yes Yes Yes

N/A N/A N/A N/A N/A 7.4 cm 4 cm  4 cm 2 cm  2 cm

N/A Excision N/A Excision N/A No No Partial resection

N/A No N/A Steroid N/A IFN Steroid, IFN Steroid, vincristine

N/A ANED N/A DOD N/A N/A ANED ANED

N/A 6.5 N/A 0.5 yr N/A N/A 1 yr N/A

18 [14] 19 [15]

14 mo 10 mo

F F

Yes Yes

Larynx Tonsil Internal auditory canal Nasal cavity, ethmoid sinus Thyroid, ipsilateral neck node Palate Scalp Neck Temporal bone, neck External auricle Face Neck Face, neck Neck Face, neck Neck Temporal bone, scalp Mandible Neck

8 cm  6 cm N/A

Radion therapy Embolization

Steroid, IFN IFN

ARD ARD

3 yr 4 yr

[12] [4] [4] [4]

10 11 12 13 14 15 16 17

N/A 3 cm  3 cm

yr yr yr yr

KMS: Kasabach–Merritt syndromes; ANED: alive with no evidence of disease; ARD: alive with residual disease; DOD: dead of disease; IFN: interferon. a Present paper.

a hypervascular tumor [8]. Despite these reported imaging findings, KHE appearance may be variable, particularly in relation to the tumor size. Also a small lesion in its early stage may be mistaken for a nonspecific benign-looking tumor [1]. Eighteen previously reported cases of otorhinolaryngologic KHE are described in Table 1. Several patients had regional lymphadenopathies at diagnosis or during the course of the disease process. Although numerous treatment modalities have been used, the treatment of choice for KHE has been complete surgical excision, occasionally combined with radiation [6,8]. Medical therapy was used for lesions that could not be completely excised or when Kasabach– Merritt syndrome was present. Medical treatment with the use of interferon, prednisolone, or vincristine has also been applied with some effectiveness [3,4]. However, these treatments had to be continued for many months even after resolution of the tumor. This case of a KHE arising in a child at an unusual site illustrates the difficulties in making the right diagnosis using imaging techniques and biopsy. In the event of hemoptypsis, KHE should also be taken into account in the differential diagnosis.

Acknowledgements None.

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