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Open access

Skull metastases from extramammary Paget’s disease emerging 8 years after initial treatment with no local progression: illustrative case

Taku Inada, Takuya Nakakuki, Norio Nakajima, Hidenori Miyake, Shinsuke Shibuya, Takashi Sakamoto, and Makiko Ishikawa

BACKGROUND

The foci of distant metastasis from extramammary Paget’s disease (EMPD) are the lung, liver, truncal bones, vertebrae, and brain. However, skull metastases have not been reported.

OBSERVATIONS

The authors treated a patient with calvarial and skull base metastases from EMPD who had undergone wide local resection of EMPD 8 years before, and they report his clinical course.

LESSONS

Because EMPD with distant metastasis is fatal, it should be recognized that EMPD can metastasize to the skull even when it seemed to be in remission for several years.

Open access

Gamma Knife radiosurgery for cluster-tic syndrome unresponsive to medical treatment: illustrative case

Lina R. Barzaghi, Edoardo Pompeo, Luigi Albano, Antonella Del Vecchio, and Pietro Mortini

BACKGROUND

Cluster-tic syndrome is a disorder characterized by the coexistence of symptoms related to both cluster headache and trigeminal neuralgia. Etiopathogenesis is not yet well defined. Medical treatment, including drugs for both cluster headache and trigeminal neuralgia, is the first therapeutic choice, whereas more invasive treatments are indicated in the case of pharmacological therapy failure or in the presence of drug side effects. To date, no randomized and/or large cohort trials describing Gamma Knife radiosurgery (GKRS) for cluster-tic syndrome are available, probably due to the syndrome’s rarity.

OBSERVATIONS

The authors describe the case of a 76-year-old woman with refractory cluster-tic syndrome who underwent GKRS with double target (the retrogasserian portion of the trigeminal nerve and the sphenopalatine ganglion). The Numerical Rating Scale (NRS) of pain and the Barrow Neurological Institute (BNI) pain intensity score before treatment were 7 (up to 10 during paroxysmal pain attacks) and V, respectively. At last follow-up, 24 months after GKRS, the patient had discontinued her pain medications and NRS and BNI pain scores were 1 and I, respectively. No trigeminal sensory disorders were reported.

LESSONS

The present case shows that GKRS, in selected cases, could be an effective treatment in patients with refractory cluster-tic syndrome.

Open access

Management of brain metastasis from eccrine porocarcinoma: illustrative case

Ryuichi Noda, Tomohiro Inoue, Sho Tsunoda, Masafumi Segawa, Yoshio Masuda, Teppei Morikawa, and Atsuya Akabane

BACKGROUND

Eccrine porocarcinoma (EP) is a rare malignant skin neoplasm, and there are still many unknowns regarding its natural history and treatment. Due to its scarcity, associated brain metastasis is a far rarer condition.

OBSERVATIONS

A 71-year-old-woman with a history of EP was diagnosed with brain metastasis. In her clinical course, the patient underwent tumor removal surgery twice and Gamma Knife radiosurgery (GKRS) four times. The tumor showed a good response to radiotherapy. The histopathological findings of the brain tumor were consistent with those of the primary skin tumor.

LESSONS

There are only a few case reports referring to the detailed treatment, especially with GKRS, of brain metastasis from EP. Few reports have presented a detailed histopathological comparison between the primary skin lesion and the metastatic brain lesion. Herein, the authors have described the clinical course, histological features, and results of multidisciplinary treatment for brain metastasis of EP.

Open access

Long-term survival in patients with long-segment complex meningiomas occluding the dural venous sinuses: illustrative cases

Zhishuo Wei, Arka N. Mallela, Andrew Faramand, Ajay Niranjan, and L. Dade Lunsford

BACKGROUND

Invasive sagittal sinus meningiomas are difficult tumors to cure by resection alone. Stereotactic radiosurgery (SRS) can be used as an adjuvant management strategy to improve tumor control after incomplete resection.

OBSERVATIONS

The authors reported the long-term retrospective follow-up of two patients whose recurrent parasagittal meningiomas eventually occluded their superior sagittal sinus. Both patients underwent staged radiosurgery and fractionated radiation therapy to achieve tumor control that extended to 20 years after their initial surgery. After initial subtotal resection of meningiomas that had invaded major cerebral venous sinuses, adjuvant radiosurgery was performed to enhance local tumor control. Over time, adjacent tumor progression required repeat SRS and fractionated radiation therapy to boost long-term tumor response. Staged multimodality intervention led to extended survival in these patients with otherwise unresectable meningiomas.

LESSONS

Multimodality management with radiosurgery and fractionated radiation therapy was associated with long-term survival of two patients with otherwise surgically incurable and invasive meningiomas of the dural venous sinuses.

Open access

Three decades of progress from surgery to medical therapy for isolated neuroaxis BRAF V600E–positive Langerhans cell histiocytosis management: illustrative case

Nallammai Muthiah, Kamil W. Nowicki, Jennifer L. Picarsic, Michael P. D’Angelo, Daniel F. Marker, Edward G. Andrews, Edward A. Monaco III, and Ajay Niranjan

BACKGROUND

“Langerhans cell histiocytosis” (LCH) is a term that encompasses single-system or multisystem disorders traditionally characterized by a proliferation of clonal CD1a+/CD207+ myeloid-derived histiocytes. In most cases of LCH, mitogen-activated protein kinase (MAPK) pathway somatic mutations lead to near universal upregulation of phosphorylated extracellular signal-regulated kinase expression. The clinical manifestations of LCH are numerous, but bone involvement is common. Intracranial lesions, especially as isolated manifestations, are rare.

OBSERVATIONS

The authors presented the case of a long-term survivor of exclusive intracranial LCH that manifested with isolated craniofacial bone and intraparenchymal central nervous system recurrences, which were managed with 3 decades of multimodal therapy. The patient was initially diagnosed with LCH at age 2 years, and the authors documented the manifestations of disease and treatment for 36 years. Most of the patient’s treatment course occurred before the discovery of BRAF V600E. Treatments initially consisted of chemotherapy, radiosurgery, and open resections for granulomatous LCH lesions. Into young adulthood, the patient had a minimal disease burden but still required additional radiosurgical procedures and open resections.

LESSONS

Surgical treatments alleviated the patient’s immediate symptoms and allowed for tumor burden control. However, surgical interventions did not cure the underlying, aggressive disease. In the current era, access to systemic MAPK inhibitor therapy for histiocytic lesions may offer improved outcomes.

Open access

Intraoperative MRI for the microsurgical resection of meningiomas close to eloquent areas or dural sinuses: patient series

Constantin Tuleasca, Rabih Aboukais, Quentin Vannod-Michel, Xavier Leclerc, Nicolas Reyns, and Jean-Paul Lejeune

BACKGROUND

Meningiomas are the most commonly encountered nonglial primary intracranial tumors. The authors report on the usefulness of intraoperative magnetic resonance imaging (iMRI) during microsurgical resection of meningiomas located close to eloquent areas or dural sinuses and on the feasibility of further radiation therapy.

OBSERVATIONS

Six patients benefited from this approach. The mean follow-up period after surgery was 3.3 (median 3.2, range 2.1–4.6) years. Five patients had no postoperative neurological deficit, of whom two with preoperative motor deficit completely recovered. One patient with preoperative left inferior limb deficit partially recovered. The mean interval between surgery and radiation therapy was 15.8 (median 16.9, range 1.4–40.5) months. Additional radiation therapy was required in five cases after surgery. The mean preoperative tumor volume was 38.7 (median 27.5, range 8.6–75.6) mL. The mean postoperative tumor volume was 1.2 (median 0.8, range 0–4.3) mL. At the last follow-up, all tumors were controlled.

LESSONS

The use of iMRI was particularly helpful to (1) decide on additional tumor resection according to iMRI findings during the surgical procedure; (2) evaluate the residual tumor volume at the end of the surgery; and (3) judge the need for further radiation and, in particular, the feasibility of single-fraction radiosurgery.