Recurrent skull base rhabdoid meningioma in an adolescent patient
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.193817
Source of Support: None, Conflict of Interest: None
Meningiomas are slow-growing lesions arising from the meningeal coverings of the brain and spinal cord. Although most meningiomas are benign, histologically distinct subsets are associated with a high risk of recurrence, especially those composed histologically of rhabdoid cells. Here, we present a case of a 16-year-old boy, suffering from a third recurrence of a frontoparietal extra-axial mass, diagnosed on histopathology as a rhabdoid meningioma (World Health Organization [WHO] Grade III).
A young boy, aged 16 years, presented with headache and a swelling over the scalp for 4 months. Computed tomography (CT) scan showed a convexity meningioma over the left frontoparietal region. Complete excision of the tumor along with dura and involved bone was done. The histology report was suggestive of an atypical meningioma (WHO grade II).
After 12 months, the boy again started having headaches and there was recurrence of the swelling, which rapidly increased in size to involve the left side of the face and neck. Re-exploration was done with complete excision of the tumor along with the adjacent dura and overlying calvarium. Cranioplasty was done with bone cement. The histology was again reported to be an atypical meningioma. He was given external beam radiation with 30 Gy/16 fractions delivered to the tumor bed.
Nine months later, the swelling recurred. This time it was very painful and extended into the neck [Figure 1]a,[Figure 1]b,[Figure 1]c. He needed opiates to relieve his intractable facial pain, which was not responding to carbamazepine. On examination, a large mass was seen involving the left frontotemporal region, left side of face, and submandibular region. The swelling was tender on deep palpation, nonfluctuant, nontransilluminant, and without cough impulse or vascular bruit. Magnetic resonance imaging (MRI) of the brain and neck showed the left frontoparietal extra-axial tumor with extension into the intratemporal fossa, pterygopalatine fossa, and submandibular region [Figure 2].
After reopening of the cranioplasty flap, excision of the left temporal meningioma was done. The meningioma was seen extending to the pterygopalatine fossa and upper part of the neck. Lower branches of the facial nerve were engulfed by the tumor and were resected. It was dissected from the great vessels of the neck. The tumor was seen to be invading the posterior wall of the left maxilla as well as the ramus of the mandible. It was removed en mass from the temporal fossa, the pterygopalatine fossa along with the ramus, and part of the body of the mandible and cheek. Reconstruction of the faciomaxillary defect was done with rectus abdominis muscle graft by performing a microvascular anastomosis along with associated skin grafting. Following this, closure of the abdominal wound was performed.
On gross examination, the specimen was a partly skin covered soft tissue mass measuring 12 × 10 × 7 cm. On slicing, soft, fleshy, necrotic areas were noted. A part of the mandible and the maxilla bearing 3 teeth each and a salivary gland were identified [Figure 3]. Multiple sections taken from this cranio-maxillo-facial mass showed an architectural pattern resembling a sarcomatous morphology. The tumor cells were oval-to-elongated, and in focal areas, cells with eccentric vesicular nuclei with prominent nucleoli and abundant eosinophilic cytoplasm imparting a rhabdoid appearance were present. Several mitotic figures were present, with an MIB1 index of >10%. The tumor also involved the subcutaneous tissues. The neoplastic cells were strongly positive for cytokeratin (AE1/AE3) and vimentin and focally positive for S-100 and epithelial membrane antigen (EMA). The neoplastic cells were negative for human melanoma black 45 (HMB-45), synaptophysin, smooth muscle actin (SMA), desmin and glial fibrillary acidic protein (GFAP), and progesterone receptor (PR). Staining for integrase interactor 1 (INI1) was present. The diagnosis of rhabdoid meningioma, WHO Grade III was made [Figure 4] and [Figure 5].
The patient had partial necrosis of the submandibular flap, which was managed with daily sterile saline dressing and intravenous antibiotics according to pus swab and culture and sensitivity report, and wound debridement was also done. The left temporal subgaleal cerebrospinal fluid (CSF) collection was managed with therapeutic CSF drainage via a lumbar drain, which was kept for 4 days. Debridement of the facial wound and split skin graft was done subsequently. The wound healed reasonably, and apart from a small pseudomeningocele at the site of the temporal craniectomy (where no cranioplasty had been done this time), the recovery was uneventful thereafter. The patient was doing well at an 11-month follow-up, with no clinical or radiological signs of recurrence [Figure 1]b and [Figure 6]. Oncological consultation has suggested a wait-and-watch attitude for the moment.
Meningiomas account for approximately 30% of all primary brain tumors, with an adjusted annual incidence of approximately 4.5 per 100,000 individuals. These tumors are most commonly reported in elderly patients, with a peak incidence in the seventh decade of life with a female-to-male ratio of about 3:1.
Most meningiomas are slow-growing benign lesions and are typically associated with the symptoms of gradually increasing intracranial pressure. The WHO classification divides the meningiomas into three categories, viz. Grade I with a low risk of recurrence or growth; Grade II, which includes atypical, chordoid, and clear cell variants; and Grade III, which is considered anaplastic and includes the rhabdoid and papillary subtypes.
The Grade III meningiomas account for 1–3% of all meningioma cases. Anaplastic meningiomas are associated with recurrence rates of up to 50–80% after surgical resection and the median survival is less than 2 years. Histologically, anaplastic meningiomas have features of malignancy with a mitotic index of 20 or more mitoses per ten microscopic high-power fields. Some anaplastic meningiomas are difficult to recognize as meningothelial neoplasms as they can resemble a sarcoma, carcinoma, or melanoma.
Rhabdoid meningioma is derived from the arachnoidal epithelial cells and contains cells resembling rhabdomyoblasts, i.e., with abundant eosinophilic cytoplasm, eccentrically placed nuclei, and paranuclear inclusions that ultrastructurally correspond to whorled bundles of intermediate filaments.
Rhabdoid meningiomas form 0.004% of all meningiomas. The male:female incidence ratio is 26:34. It occurs over a wide age range, 2–84 years, but is most common among teenagers. The recurrence rate is nearly 87% with a mortality of approximately 53%. In 1998, Kepes et al., and Perry et al., described the first two series of meningiomas with rhabdoid transformation. These meningiomas often recur, and the rhabdoid features become more apparent in subsequent biopsies, which may possibly explain why the diagnosis was missed on previous biopsies in our case.
The most commonly used immunomarker for meningioma is EMA. Vimentin staining shows a diffuse cytoplasmic positivity; however, both these markers lack specificity. Expression of the progesterone receptor is inversely associated with the meningioma grade. The rhabdoid meningiomas show a strong positivity for cytokeratin in a perinuclear cytoplasmic hof-like fashion, as has been demonstrated in our case. EMA and S-100 are variably positive. The usual muscle markers such as desmin are negative. Unlike rhabdoid tumors elsewhere, INI1 expression is retained.
The term malignant rhabdoid tumor (MRT) was first used to describe a distinctive pediatric renal tumor. The term was then extended to similar extrarenal pediatric tumors (extrarenal MRT), including the atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system. These tumors commonly show mutation of the INI1 gene on 22q11.2. It is a member of the switch 1/sucrose non-fermentable (SWI/SWF) chromatin-remodelling complex and is normally expressed in all tissues. INI1 is found to be a useful marker in central nervous system primitive neuroectodermal tumors, alveolar rhabdomyosarcoma, desmoplastic small round cell tumor, childhood glioblastoma multiforme, childhood anaplastic astrocytoma, etc., The rhabdoid meningiomas, however, do not show this characteristic loss of INI-1 staining, suggesting that they are different from the usual family of rhabdoid tumors.
A rhabdoid phenotype has also been described in a variety of tumors with a different histogenesis, including carcinomas, sarcomas, gliomas, and melanomas (composite extrarenal rhabdoid tumors or CERT). These tumors do not commonly show 22q11.2 deletions. The rhabdoid morphology is related to an aggressive behaviour and a poor outcome, irrespective of the tumor histogenesis. Loss of protein 4.1B (DAL-1) expression is a common aberration detected in meningiomas of all WHO grades, especially in higher grade forms; however, this has not been reported in rhabdoid meningiomas.
Rhabdoid meningiomas are notorious for the involvement of the contiguous structures such as important vessels and nerves. A list of prior published cases from our country is summarized in [Table 1].,,, Hence, complete resection of the tumor becomes difficult. Due to the rarity and aggressive nature of the lesion, limited data is available regarding its incidence and follow up, leading to controversies and disagreement regarding the ideal management protocol.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]